Abstract
RATIONALE: Pilocytic astrocytoma (PA) typically shows biphasic pattern with a mixture of loose microcystic and compact regions, in which it is not uncommon to see heterogeneous morphology. However, there has not been reported in the literatures of the PA type that shows similarity to dysembryoplastic neuroepithelial tumor (DNT) in both histological morphology and immunophenotype.
PATIENT CONCERNS: The present study described a case of PA affecting the right temporal-occipital lobe in a 22-year-old male patient. Morphologically, it composed of totally distinctive microcystic pattern. The classical biphasic pattern of PA was not observed. Immunohistochemically, neuronal marker NeuN was expressed in tumor cells scattered in the background which simulated its expression morphology in DNT. However, KIAA1549-BRAF fusion gene was identified by fluorescence in situ hybridization (FISH), supporting for the diagnosis of PA.
DIAGNOSES: DNT-like PA (WHO grade I).
INTERVENTIONS: The tumor was totally removed via a right temporal-occipital craniotomy.
OUTCOMES: The patient is free of local recurrence and dissemination eleven months after surgical resection of the lesion.
LESSONS: We herein report a rare case of DNT-like PA. For diagnosis, KIAA1549-BRAF fusion gene should be detected under similar situation.

Infantile medulloepithelioma in the lateral ventricle and cerebellopontine angle: Two case reports.

Medicine (Baltimore). 2018 May;97(20):e10751

Authors: Li Q, Chen N, Ju Y

Abstract
RATIONALE: Intracranial medulloepitheliomas are extremely rare and highly malignant. Intraventricular medulloepitheliomas are even rarely reported, and little is known about the clinical features.
PATIENT CONCERNS: In this article, we report two cases of intracranial medulloepitheliomas. In the first patient, a one-month old boy, the tumor was located in in right lateral ventricle, which was the first report of such location of this disease; in the second patient, an eleven-month old girl, the tumor was in right cerebellopontine angle.
DIAGNOSES: Both patients were diagnosed as medulloepithelioma by pathologists.
INTERVENTIONS: Both patients underwent craniotomy to resect the lesion totally.
OUTCOMES: The boy underwent chemotherapy after operation and was alive 3 months after operation. The girl died 6 months after operation, despite aggressive adjuvant chemotherapy.
LESSONS: Surgical resection is safe and effective to prolong patient survival. However, despite aggressive adjuvant therapy, prognosis of medulloepithelioma remains poor, and further study is needed to improve treatment of this rare disease.

Abstract
The anterior cingulate gyrus (ACG) is a continued focus of research as its exact role in brain function and vast connections with other anatomical locations is not fully understood. A review of the literature illustrates the role the ACG likely plays in cognitive and emotional processing, as well as a modulating role in motor function and goal-oriented behaviors. While lesions of the cingulate gyrus are rare, each new case broadens our understanding of its role in cognitive neuroscience and higher order processing. The authors present the case of an 8-year-old boy with a 1-month history of staring spells, agitated personality, and hyperphagia notable for the consumption of paper, who was found to have a 3-cm tumor in the left ACG. Following surgical resection of the tumor, his aggressive behavior and pica were ameliorated and the patient made an uneventful recovery, with no evidence of recurrence over the last 6 years since surgical resection. Here we discuss a unique behavioral presentation of pica, along with a review of the current literature, to illustrate functions of the ACG relevant to the location of the lesion.

Abstract
A 26-year-old female presented with vision loss accompanied by migraine-like headaches. A contrast-enhanced magnetic resonance imaging of the brain was performed which revealed findings suggestive of stroke-like migraine attacks after radiation therapy (SMART) syndrome. SMART syndrome is a delayed complication of brain radiation characterized by neurologic symptoms including migraine-like headaches, seizures, and hemispheric impairment. The purpose of this article is to make the readers aware of this rare complication of brain irradiation. Appropriate diagnosis of SMART syndrome is essential to avoid invasive tests.

Treatment Outcome in Patients with Primary Central Nervous System Germ Cell Tumour: Clinical Experience from a Regional Cancer Centre in North India.

Pediatr Neurosurg. 2017;52(4):240-249

Authors: Biswas A, Julka PK, Bakhshi S, Singh M, Rath GK

Abstract
BACKGROUND: Primary intracranial germ cell tumour is a rare entity and constitutes 2-3% of all paediatric brain tumours in Western countries. We herein intend to report the clinical features and treatment outcome of patients with primary central nervous system germ cell tumour treated at our institute.
METHODS: Clinical data were collected by retrospective chart review from 2006 to 2012. Histopathology slides were reviewed and relevant immunohistochemistry stains were done. Overall survival (OS) and progression-free survival (PFS) were analysed by the Kaplan-Meier product-limit method.
RESULTS: Twenty patients met the study criterion (male:female = 7:3). Median age at presentation was 13 years. Tumour location was pineal in 10 patients, suprasellar in 6, thalamic in 2, basal ganglion in 1, and spinal in 1. Leptomeningeal spread was noted in 1 patient at presentation. Surgical resection was gross-total in 7 patients (35%), near-total in 2 (10%), subtotal in 4 (20%), and limited to biopsy in 6 (30%). The tumours were germinomatous, non-germinomatous, and of mixed germ cell subtype in 17 patients (85%), 2 patients (10%), and 1 patient (5%), respectively. Systemic chemotherapy (median of 4 cycles) was given to 19 patients (95%). The common regimens used were a combination of bleomycin, etoposide and cisplatin (BEP) in 14 patients (70%) and etoposide and cisplatin (EP) in 5 patients (25%). Radiation therapy (40-50 Gy in conventional fractionation; median of 42 Gy) was delivered to 17 patients (85%): local radiation in 6 and whole ventricular, whole brain, and craniospinal irradiation followed by a boost in 5, 3, and 3 patients, respectively. After a median follow-up of 44.52 months, 17 patients (85%) were in complete response and 3 (15%) had progressive disease. Death and disease recurrence were noted in 6 patients (30%) and 1 patient, respectively. Median OS and PFS were not reached. The actuarial rates of OS at 3 and 5 years were 75.8 and 68.9%, respectively. The actuarial rates of PFS at both 3 and 5 years were 81.6%.
CONCLUSION: Multimodality treatment consisting of limited resection followed by platinum-based systemic chemotherapy and radiotherapy (40-50 Gy) is a reasonable treatment strategy in patients of primary central nervous system germ cell tumour in a developing nation.

Abstract
STUDY DESIGN: A retrospective, multicenter, case-controlled study.
OBJECTIVE: The aim of this study was to investigate the clinical and radiographical differences between thoracic idiopathic spinal cord herniation (ISCH) and spinal arachnoid cyst (SAC).
SUMMARY OF BACKGROUND DATA: ISCH and SAC are relatively rare diseases. Preoperative misdiagnose was frequently reported in both; however, these clinical and radiographical differences remain unclear.
METHODS: Of 30,469 patients who underwent spinal surgery, 18 (0.059%) and 22 (0.072%) patients were diagnosed as ISCH or SAC at nine hospitals, respectively, and their clinical and radiographical data were retrospectively evaluated. The spinal cord kink angle was measured on magnetic resonance or computed tomography myelography sagittal images; the kink angle was the exterior angle formed between the two tangents to the dorsal-side inflection points at the maximally affected level.
RESULTS: There were no significant differences in age, gender, and duration of symptoms. Preoperative motor deficit was significantly higher (94.4%) and severer (manual muscle testing: 3.1 ± 1.1) in ISCH. Brown-Séquard syndrome was observed in 38.9% of ISCH, while no patients in SAC. In addition, leg sensory disorder and bladder rectal disorder were significantly more common in ISCH, while back pain was significantly frequent in SAC. With respect to radiographical findings, the length of disease (5.1 ± 2.7 levels) and altered cerebrospinal flow (CSF) (81.8%) in the lesion was significantly longer and more common in SAC. On the contrary, the kink angle was significantly greater in ISCH (54.0 ± 23.1°) than in SAC (14.1 ± 12.0°) (P < 0.001). The cut-off value of the kink angle to distinguish ISCH and SAC was 32.8°.
CONCLUSION: Patients with ISCH commonly had severe preoperative neurological deficit, Brown-Séquard syndrome, and higher kink angle, while patients with SAC had back pain, longer length of disease, and altered CSF in the lesion.
LEVEL OF EVIDENCE: 4.

Abstract
Acromegaly is a rare, chronic, progressive disease characterized by an excess secretion of growth hormone (GH) and increased circulating insulin-like growth factor 1 (IGF-1) concentrations. It is caused by a pituitary adenoma in the vast majority of cases. The clinical diagnosis, based on symptoms related to GH excess, is often delayed due to the insidious nature of the disease. Consequently, patients often have established systemic complications at diagnosis with increased morbidity and premature mortality. Serum IGF-1 measurement is recommended as the initial screen for patients with suspected acromegaly. The gold standard diagnostic test remains the oral glucose tolerance test with concomitant GH measurement. Therapy for acromegaly is targeted at decreasing GH and IGF-1 levels, ameliorating patients' symptoms and decreasing any local compressive effects of the pituitary adenoma. The therapeutic options for acromegaly include surgery, medical therapies (such as dopamine agonists, somatostatin receptor agonists and the GH receptor antagonist pegvisomant) and radiotherapy. A multidisciplinary approach is recommended with often a requirement for combined treatment modalities. With disease control, associated morbidity and mortality can be reduced. The recently published evidence-based guidelines by the Endocrine society addressed important clinical issues regarding the evaluation and management of acromegaly. This review discusses advances in our understanding of the pathophysiology of acromegaly, diagnosis of various forms of the disease and focuses on current treatment modalities, and on future pharmacological therapies for patients with acromegaly.

Abstract
BACKGROUND: Epithelioid glioblastoma, a high-grade, diffuse astrocytic tumor variant, comprises closely packed epithelioid cells and rhabdoid cells. This rare tumor usually develops in the cerebral cortex and diencephalon; however, in the case reported here, it was located intraventricularly.
CASE DESCRIPTION: A 47-year-old woman was referred to our hospital with a right intraventricular mass that had rapidly increased in size. On discovery of the tumor 3 years earlier at the referring hospital, the mass was small, calcified, and attached to the periventricular parenchyma. Over the next 2 years, the mass grew slowly, as observed on periodic magnetic resonance imaging scans. Forty days before the referral, the patient experienced headache and nausea, and marked growth and intratumoral hemorrhage were visible on a computed tomography scan of the head. The tumor was partially removed via a superior parietal lobule corticotomy. Histopathological examination confirmed an isocitrate dehydrogenase-wild-type epithelioid glioblastoma with a BRAF V600E mutation, but the original slow-growing lesion was no longer detected. Consequently, we assume that in this case, a low-grade glioma transformed into an aggressively malignant epithelioid glioblastoma.
CONCLUSIONS: We present the first case of an intraventricular epithelioid glioblastoma that might have arisen from a low-grade glioma with calcification. We recommend including this tumor variant in the differential diagnosis of lateral ventricle tumors.

Abstract
BACKGROUND: Epidermoid tumors are rare, benign slow-growing congenital tumors, most frequently located in the cerebellopontine angle of the intracranial cavity. They usually grow to a large size before patients become symptomatic. Although these tumors are amenable to surgery, their adherence to neurovascular structures poses a surgical challenge that results in subtotal resection, thus increasing the risk of recurrence.
CASE DESCRIPTION: We report 2 adult patients whose imaging studies revealed epidermoid tumors located in the cerebellar vermis, an uncommon site for such tumors. The patients presented with variable symptomatology. We highlight the imaging features and challenges of surgery. Both patients had good outcomes, with resolution of symptoms and neurologic deficits.
CONCLUSIONS: A safe complete excision of epidermoid tumor and its capsule is possible with a good understanding of their clinical and radiologic features and a high index of suspicion. To the best of our knowledge, this is the first report of cerebellar vermian epidermoid tumors from sub-Saharan Africa.

Abstract
BACKGROUND: Primary meningeal melanocytoma is a benign lesion in the central nervous system derived from leptomeningeal melanocytes, most commonly growing in the posterior fossa and cervical spinal cord. The sellar primary meningeal melanocytoma (SPMM) is an exceptionally rare tumor. Here we report the ninth published case of an SPMM, and also discuss the problems of differential diagnosis and management of these tumors.
CASE DESCRIPTION: The patient presented with visual impairment and irregular menstruation with no other symptoms. Her general examination was otherwise unremarkable. Endocrine tests disclosed normal endocrine function except for slight hyperprolactinemia. Magnetic resonance imaging revealed a sellar lesion. The patient underwent a successful total transsphenoidal removal of the tumor without neurologic sequelae. Based on this case report, SPMM should be included in the differential diagnosis of malignant and/or metastatic melanoma.
CONCLUSIONS: SPMM is an exceptionally rare tumor. Differentiating sellar melanocytic tumors from other sellar diseases through clinical and radiologic investigations is very difficult. The treatment of choice for SPMM is complete surgical resection via a transsphenoidal approach. For patients with a partially resected tumor, radiotherapy is an effective complementary treatment modality.

Extraventricular Neurocytoma in the Left Frontal Lobe: A Case Report and Literature Review.

World Neurosurg. 2018 Apr;112:178-181

Authors: Chen F, Jin R, Wu X, Dong Z, Chen D

Abstract
BACKGROUND: Neurocytoma is a rare brain neoplasm of neuroepithelial origin that occurs predominantly in the ventricular system adjacent to the interventricular foramen and septum pellucidum. However, extraventricular neurocytoma is an extremely rare entity, with poor clinical, radiologic, and histopathological characterization. Here we report a case of an extraventricular parafalcine neurocytoma in the left frontal lobe. We also examine previously reported cases of extraventricular neurocytoma in an attempt to provide an up-to-date summary of the condition.
METHODS: A literature search was performed using PubMed with specific key terms, inclusion criteria, and exclusion criteria. Selected case studies and case series were then compared, and statistical analyses were performed where appropriate. We report a 59-year-old woman presenting with weakness in her right leg and urinary incontinence. Physical examination revealed muscle strength of grade 3/5 in the right lower extremity. Brain magnetic resonance imaging showed a parafalcine mass in the left frontal lobe, with perilesional edema; the cerebral falx and lateral ventricle were shifted due to the compression. Gross total resection was performed.
RESULTS: Histopathological examination revealed a neurocytoma. Immunohistochemical staining showed diffuse positivity for synaptophysin. MIB-1 staining for Ki-67 antibody showed a labeling index of 20%. No adjuvant radiation or chemotherapy was administered. Brain computed tomography performed at a 3-month follow-up showed no signs of recurrence.
CONCLUSION: Extraventricular neurocytoma occurring in the brain parenchyma is a very rare central nervous system tumor. Its clinical and radiologic manifestations are nonspecific. The diagnosis depends on histopathological and immunohistochemical examination. Surgical resection should be the first-choice treatment.

Development of primary central nervous system lymphoma in a systemic lupus erythematosus patient after treatment with mycophenolate mofetil and review of the literature.

Lupus. 2017 Oct;26(11):1224-1227

Authors: Balci MA, Pamuk GE, Unlu E, Usta U, Pamuk ON

Abstract
Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma and four cases of PCNSL have previously been described in association with mycophenolate mofetil. We report the fifth case of PCNSL in a patient with lupus nephropathy while on mycophenolate mofetil treatment.

Abstract
BACKGROUND: The term of "tumor-to-lesion metastasis" is an extension of "tumor-to-tumor metastasis", which is a rare but well-documented phenomenon. Tumor metastasis to the meninges and/or central nervous system (CNS) is rare in patents with multiple sclerosis (MS), although MS lesions bear many similarities to the primary tumor microenvironment and the metastatic niche. We present the first case of malignant tumor metastasis to MS lesions with immunophenotyping of inflammatory cells in the metastatic foci.
CASE DESCRIPTION: A 45-year-old male patient with a 6-year history of MS and newly diagnosed lung carcinoma developed carcinoma metastases to the meninges and CNS as well as into mixed active/inactive MS lesions. The carcinoma-hosting MS lesions exhibited abundant macrophages/microglia with ongoing demyelination but rare T-cells. In comparison, a 46-year-old female patient with a 21-month history of MS and newly diagnosed gastric carcinoma was found to have leptomeningeal carcinomatosis and separate active MS lesions containing not only frequent macrophages/microglia but also T-cells.
CONCLUSIONS: The carcinoma-hosting MS lesions are unlike typical active lesions but recapitulate the CNS metastatic niche. Our observations suggest that metastasis-hosting MS lesions might require a distinct immune microenvironment to be permissive to the seeding and growth of metastatic tumors.

Abstract
BACKGROUND: The pineal gland has been demonstrated to be involved in the development of mood and psychotic disorders. However, few studies have looked at the relationship between pineal region tumors and psychiatric disorders. Intracranial epidermoid cysts usually arise in the cerebellopontine angle area and are extremely rare in the pineal region. The case of pineal epidermoid cyst presenting as schizophrenia has never been reported before.
CASE PRESENTATION: We described the case of a 23-year-old man who presented to the hospital with symptoms suggestive of schizophrenia. During work-up, he was found to have a pineal lesion on brain magnetic resonance imaging. Total resection of the tumor was subsequently performed, and pathology confirmed an epidermoid cyst. One month after surgery, the patient's psychotic symptoms significantly improved free of drug and fully returned to work 3 months postoperatively.
CONCLUSIONS: This case highlights the importance of including mass lesions of the pineal region in the differential diagnosis of psychotic disorders. It also provides further support that the pineal region may play a role in the pathophysiology of psychiatric diseases, although more studies will be needed to elucidate this interesting connection.

Abstract
BACKGROUND: Pediatric cerebral ganglioneuroblastoma is an exceedingly rare tumor.
CASE DESCRIPTION: We describe the case of a 4-year-old boy with sudden mental status decline who was found to have a large intracranial lesion with intraventricular extension.
CONCLUSION: Management of the case and pathologic findings are discussed, along with a review of the literature on this rare entity.

Abstract
BACKGROUND: Exophytic growth (EG), wherein the tumor mass grows beyond the neuraxial boundary formed by pia mater, remains a rare pattern of glioma growth. It has been described in gliomas at several locations like the brainstem, cerebellum, suprasellar area, spinal cord, and insula. However, EG in hemispheric grade 2 astrocytomas, particularly affecting the convexity subarachnoid space, as against the more spacious basal subarachnoid space, is exceedingly rare. To the best of our knowledge, there is only 1 such case reported in the English literature. Due to the extreme rarity, little is known about these tumors, particularly the mechanisms underlying the EG and their possible clinical implications.
CASE DESCRIPTION: A 32-year-old woman presented with partial motor seizures without any neurologic signs. On magnetic resonance imaging of the head, a nonenhancing, T2-hyperintense, right frontal lobe lesion was noted. The majority of the lesion was occupying the adjacent subdural space while still remaining in continuity with the intra-axial globular component. During surgery, the exophytic nature of the lesion was confirmed. We performed maximal resection of the exophytic portion with subtotal excision of the intra-axial component. She recovered uneventfully after surgery and subsequently received external beam radiotherapy for the residual tumor.
CONCLUSION: Although extremely rare, cerebral hemispheric grade 2 astrocytomas may have an EG pattern. The exact mechanisms underlying this are not clearly known. More such cases need to be reported so that we can decipher the true nature of these tumors.

Case of Primary Central Nervous System Lymphoma Arising at Site of Remote Herpes Encephalitis.

World Neurosurg. 2018 May;113:217-222

Authors: Li V, Levine AB, Gooderham PA, Yip S, Chew J

Abstract
BACKGROUND: Primary central nervous system lymphoma (PCNSL) is a rare malignancy that usually arises in the context of severe immunosuppression but has incompletely understood etiology, limiting effective treatments.
CASE DESCRIPTION: We present the case of an 81-year-old immunocompetent man who developed a PCNSL in the right temporal lobe, at the site of a remote episode of herpes simplex virus (HSV) encephalitis 8 years prior. There are numerous viruses with known oncogenic associations; however, to our knowledge, this is the first reported case of PCNSL with an antecedent HSV infection.
CONCLUSIONS: We discuss this case in the context of our current understandings of the pathogenesis of HSV encephalitis and PCNSL and postulate mechanisms through which the 2 could be associated.

Behavior Changes and Gait Unsteadiness: The Value of Imaging and Prompt Neurosurgical Intervention.

Acta Med Port. 2017 Jan 31;30(1):77-79

Authors: Costa A, Marques-Matos C, Reis C, Carvalho M, Pinto M

Abstract
Cavernous angiomas are central nervous system malformations. Most common manifestations are seizures and acute focal neurological deficits. We present a case report of a seventy-one year-old man with a two-month history of behavior changes, attention deficit and indifference followed by gait unsteadiness. Neuropsychological evaluation showed severe cognitive impairment and executive dysfunction. Head computed tomography depicted a supraventricular hydrocephaly. Magnetic resonance imaging revealed a small hemorrhage, contiguous to a mesencephalic cavernous angioma, obstructing the Sylvius aqueduct, causing secondary hydrocephalus. Four months after endoscopic ventriculocisternostomy, neuropsychological evaluation showed improvement and the patient regained autonomy. Parenchyma cavernous angiomas causing direct hemorrhage and subsequent obstruction of the Sylvian aqueduct are uncommon. Sub-acute behavior and mental state abnormalities are rare first manifestations of cavernous angioma and requires high clinical suspicion for its correct diagnosis. Magnetic resonance imaging evaluation is crucial in the detection of such patients as prompt neurosurgical intervention may substantially improve cognitive function.

Abstract
Diffuse leptomeningeal glioneuronal tumors (DLGNT) represent rare CNS neoplasms which have been included in the 2016 update of the WHO classification. The wide spectrum of histopathological and radiological features can make this enigmatic tumor entity difficult to diagnose. In recent years, large-scale genomic and epigenomic analyses have afforded insight into key genetic alterations occurring in multiple types of brain tumors and provide unbiased, complementary tools to improve diagnostic accuracy. Through genome-wide DNA methylation screening of > 25,000 tumors, we discovered a molecularly distinct class comprising 30 tumors, mostly diagnosed histologically as DLGNTs. Copy-number profiles derived from the methylation arrays revealed unifying characteristics, including loss of chromosomal arm 1p in all cases. Furthermore, this molecular DLGNT class can be subdivided into two subgroups [DLGNT methylation class (MC)-1 and DLGNT methylation class (MC)-2], with all DLGNT-MC-2 additionally displaying a gain of chromosomal arm 1q. Co-deletion of 1p/19q, commonly seen in IDH-mutant oligodendroglioma, was frequently observed in DLGNT, especially in DLGNT-MC-1 cases. Both subgroups also had recurrent genetic alterations leading to an aberrant MAPK/ERK pathway, with KIAA1549:BRAF fusion being the most frequent event. Other alterations included fusions of NTRK1/2/3 and TRIM33:RAF1, adding up to an MAPK/ERK pathway activation identified in 80% of cases. In the DLGNT-MC-1 group, age at diagnosis was significantly lower (median 5 vs 14 years, p < 0.01) and clinical course less aggressive (5-year OS 100, vs 43% in DLGNT-MC-2). Our study proposes an additional molecular layer to the current histopathological classification of DLGNT, of particular use for cases without typical morphological or radiological characteristics, such as diffuse growth and radiologic leptomeningeal dissemination. Recurrent 1p deletion and MAPK/ERK pathway activation represent diagnostic biomarkers and therapeutic targets, respectively-laying the foundation for future clinical trials with, e.g., MEK inhibitors that may improve the clinical outcome of patients with DLGNT.

Abstract
BACKGROUND: Rhabdomyosarcoma (RMS), one of the most common soft tissue sarcomas of childhood, is very rare in the neonatal period (0.4-2% of cases). In order to gain a deeper understanding of this disease at such age, patient and tumor features, as well as treatment modality and outcome need to be reported.
CASE PRESENTATION: We describe two cases with congenital RMS treated at Bambino Gesù Children's Hospital between 2000 and 2016. They represent only 2.24% of all RMS patients diagnosed during that period in our Institution; this data is in agreement with the incidence reported in the literature. They reflect the two different clinical forms in which the disease may manifest itself. One patient, with the alveolar subtype (positive for specific PAX3-FOXO1 fusion transcript) and disseminated disease, had a fatal outcome with central nervous system (CNS) progression despite conventional and high dose chemotherapy. The other child, with the localized embryonal subtype, was treated successfully with conservative surgery and conventional chemotherapy, including prolonged maintenance therapy. He is disease free at 7 years of follow-up.
CONCLUSIONS: RMS can also be diagnosed during the neonatal period. Given the young age, disease management is often challenging, and especially for the alveolar subtype, the outcome is dismal despite intensified multimodality therapy. In fact, it characteristically manifests with multiple subcutaneous nodules and progression most commonly occurs in the CNS (Rodriguez-Galindo et al., Cancer 92(6):1613-20, 2001). In this context, CNS prophylaxis could play a role in preventing leptomeningeal dissemination, and molecular studies can allow a deeper tumor characterization, treatment stratification and identification of new potential therapeutic targets.

Abstract
RATIONALE: Pituitary apoplexy (PA) is a syndrome caused by acute hemorrhage or infarction of the pituitary gland, generally within a pituitary adenoma. PA following spinal surgery is a very rare complication and may be difficult to diagnose. However, early diagnosis of PA is essential for the timely treatment of pan-hypopituitarism and prevention of severe neurologic complications.
PATIENT CONCERNS: A 73-year-old man had a posterior lumbar fusion surgery over a period of 8 hours on prone position. The patient complained of severe intractable headache accompanied by ophthalmalgia and ptosis on right eye 2 days after the surgery.
DIAGNOSIS: Brain magnetic resonance imaging revealed a 1.3 × 2.6 × 2 cm mass in the sellar fossa and suprasellar region and the laboratory tests indicated pan-hypopituitarism.
INTERVENTIONS: High-dose intravenous steroid therapy and trans-sphenoidal hypophysectomy were performed.
OUTCOMES: Pathological evaluation of the surgical specimen revealed a pituitary adenoma with total necrosis, indicating that the PA occurred because of tumor infarction. The patient recovered fully after resection of the pituitary adenoma and hormonal therapy.
LESSONS: Even though the incidence is low, PA has been related to blood pressure fluctuations or vasospasm during surgery. PA should be considered during differential diagnosis in cases of postoperative severe headache or ophthalmic complications.

Abstract
BACKGROUND: Oligodendrogliomas constitute 5% of all primary brain tumors and are the third most common cancer among intracranial tumors. More than 90% of oligodendrogliomas have supratentorial localization. Oligodendrogliomas arising in cerebellar peduncle are substantially rare, <1%. Up to now, 6 oligodendroglioma cases with cerebellar peduncle localization have been presented. We aimed to discuss our World Health Organization grade II oligodendroglioma case, which originated from cerebellar peduncle in the light of literature.
CASE DESCRIPTION: We report a case of 43-year-old woman. Her complaints were headache, perioral numbness and gait abnormality. Magnetic resonance imaging revealed cyst-like well-demarcated lesion localized in right cerebellar peduncle. Tumor excision was performed by turning around the tumor.
CONCLUSION: It should be kept in mind that oligodendrogliomas may be present as cerebellar peduncle localized tumors.

Abstract
OBJECTIVE: Primary intracranial angioleiomyoma is a rare and distinct neoplasm. Only 29 cases have been reported previously, and we aimed to investigate the clinical and radiopathologic features of these lesions.
METHODS: Medical records and radiographs of 8 patients (7 male and 1 female; mean age: 48.7 years) at our institution were reviewed retrospectively. Patient follow-up and a literature review were performed.
RESULTS: The most common preoperative symptom was a visual defect (n = 2), followed by diplopia (n = 1) and abducens paralysis (n = 1). Three patients were asymptomatic. The parasellar area (particularly the cavernous sinus) was the predilection site (n = 4; 50.0%). Radiographically, all lesions were solid without cystic degeneration. All lesions appeared with T1 hypointensity and T2 hyperintensity, and they were gradually heterogeneously enhanced after the administration of gadolinium. Complete resection was achieved in 7 patients (87.5%) without recurrence after 26.8 months of follow-up. Mitosis was rarely observed, and the Ki-67 labeling index was less than 1%; pathologically, the cavernous type was the most common.
CONCLUSIONS: Primary intracranial angioleiomyomas were prevalent in middle-aged men, and they usually involved the cavernous sinus and were frequently pathologically identified as the cavernous type. Preoperative symptoms varied depending on lesion location. The preoperative diagnosis of primary intracranial angioleiomyomas is difficult without pathology. Digital subtraction angiography and preoperative embolization are useful for differential diagnosis and surgery. Given the indolent biology of these tumors, a favorable outcome can be achieved using total resection without recurrence. A larger sample size with long-term follow-up is needed to verify our findings.

Intracranial Erdheim-Chester Disease Mimicking Parafalcine Meningioma: Report of Two Cases and Review of the Literature.

World Neurosurg. 2018 Feb;110:365-370

Authors: Wagner KM, Mandel JJ, Goodman JC, Gopinath S, Patel AJ

Abstract
BACKGROUND: Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis that typically occurs in middle-aged patients. It is usually characterized by multifocal osteosclerotic lesions of the long-bones, however many cases have extraskeletal involvement. Central nervous system (CNS) involvement is common, but isolated CNS involvement at presentation has rarely been reported.
CASE DESCRIPTION: Here we report two cases of dural-based ECD mimicking meningioma on imaging with no other identified sites of disease.
CONCLUSION: ECD is a rare disease, with isolated CNS involvement reported only a few times in the literature. The significance of this presentation requires additional study and long-term follow up.

Abstract
BACKGROUND: Hypertrophic olivary degeneration (HOD) is a rare phenomenon in the dento-rubro-olivary pathway caused by lesion or disruption of the fibers of the Guillain-Mollaret triangle. Hemorrhage of pontine and midbrain cavernous angiomas can rarely lead to HOD portending neurologic deterioration and possible concomitant life-threatening complications; for this reason, it may define a poignant consideration in planning intervention.
CASE DESCRIPTION: The patient was a 57-year-old woman with known midbrain-pontine cavernous angioma. For several years, the lesion had been stable, as shown by imaging follow-up, until 10 months before the patient presented with falls, dysarthria, and headache. Imaging showed some decrease in size as well as blood product around the cavernous angioma, suggesting interim period hemorrhage and interval development of HOD.
CONCLUSIONS: The literature regarding imaging recommendations for stable cavernous angioma in the midbrain-pontine junction is reviewed. The implication of HOD for patient outcome is discussed and a comment is made on how the development of HOD may affect management of the cavernous angioma.

Abstract
Cushing disease is a relatively rare cause of Cushing syndrome secondary to a hyperfunctioning pituitary adenoma. In addition to signs and symptoms of hypercortisolism, Cushing disease may present with diverse otolaryngic manifestations, which may guide diagnosis and management. We performed a retrospective chart review of patients who were found to have Cushing disease and who underwent transnasal transsphenoidal surgery for pituitary adenomas between January 1, 2007, and July 1, 2014, at a tertiary academic medical center. There were 37 consecutive patients in this series with Cushing disease caused by a pituitary adenoma. Fifteen (41%) patients complained of visual changes. Five (14%) patients suffered from obstructive sleep apnea. Four (11%) patients had thyroid disease. Other symptoms included hearing loss, vertigo, tinnitus, epistaxis, dysphagia, and salivary gland swelling. Although Cushing disease traditionally presents with classic "Cushingoid" systemic features, it also may present with various otolaryngic manifestations. A thorough workup by otolaryngologists is critical in the comprehensive management of these patients.

Abstract
OBJECTIVE: Intracranial and intraspinal clear cell meningiomas (CCMs) are rarely reported because of their extremely low incidence, and the current understanding of CCM is poor. The purpose of this study was to analyze the incidence and the clinical, radiologic, pathologic, and prognostic features of intracranial and intraspinal CCMs.
METHODS: Among 14,310 cases of intracranial and intraspinal meningiomas that were surgically treated between 2006 and 2016 at Beijing Tian Tan Hospital, 56 were chosen for analysis and retrospectively reviewed. To determine which parameters were associated with longer progression-free survival (PFS) and overall survival (OS), statistical analysis was performed.
RESULTS: CCMs accounted for approximately 0.39% of all intracranial and intraspinal meningiomas. Patients with CCM had a mean age of 32.3 years and there was a female predilection (20 males and 36 females). Gross total resection was achieved in 35 cases, and subtotal resection was achieved in 21 cases. All patients were followed up for 10-206 months after surgery. Twenty-six patients experienced tumor recurrence, and the median PFS was 48.0 months. The 1-year, 3-year, and 5-year PFS was 87.5%, 59.8%, and 41.8%, respectively. Twelve patients died of tumor recurrence, and the median OS was not available. The 1-year, 3-year, and 5-year OS was 98.2%, 91.3%, and 65.8%, respectively. Univariate analysis showed that total tumor removal was significantly associated with a better prognosis. Multivariate analysis confirmed only Simpson grade III and IV resection as an independent risk factor for shorter PFS. Radiotherapy mildly improved PFS after both gross total resection and subtotal resection, showing no significant difference because of the small sample size and short follow-up duration.
CONCLUSIONS: CCM is a rare subtype of World Health Organization grade II meningioma. CCM typically involves young patients and shows a female predilection and high recurrence rate. When possible, total resection is the primary and most suitable treatment for CCM. For patients with primary tumors, radiotherapy is recommended after the initial operation regardless of the extent of resection. For patients with disease recurrence, secondary surgery combined with radiotherapy might serve as an effective treatment.

Abstract
BACKGROUND: Solitary intracranial metastases of differentiated thyroid carcinoma (DTC) are rare, and their management is still controversial. This research aimed to seek suitable therapeutic methods for this disease.
METHODS: We retrospectively studied 11 pathologically confirmed cases of solitary intracranial metastases of DTC in a single institution from January 2000 to December 2016 and systematically reviewed 52 cases of this disease out of 416 cases described in the literature on PubMed. These 63 cases were analyzed by Kaplan-Meier analysis, Cox regression analysis, and post-hoc test.
RESULTS: The diameters of intracranial metastases in all 63 cases were greater than 2 cm. Lung metastases (P = 0.000) and neurosurgery (P = 0.014) were 2 independent prognostic factors of this disease. Only neurosurgery (P = 0.05) was an independent prognostic factor in the patients with lung metastases, and neurosurgery (P = 0.044) and whole brain radiotherapy (WBRT) (P = 0.041) were 2 independent factors in the patients without lung metastases. Longer overall survival (OS) was achieved in the gross total removal (GTR) and subtotal removal (STR) groups than in the no neurosurgery group (P = 0.015, P = 0.084, respectively), and there was no significance between the GTR and STR groups (P = 0.918).
CONCLUSION: The patients without lung metastases had a better prognosis than did the patients with lung metastases. Neurosurgery could obviously improve the prognosis, and if possible, GTR of metastases was supposed to be achieved; STR could be considered. WBRT was a suitable method after neurosurgery in the patients without lung metastases but could not prolong OS in the patients with lung metastases.

Abstract
BACKGROUND: Ependymomas are rare central nervous system tumors. Local tumor distribution in the central nervous system depends on age: among adults, ependymomas occur mostly in the spinal cord, whereas among children, intracranial manifestations are more common. To date, there are no prospective studies about treatment strategies for ependymomas. In most cases, complete tumor resection is recommended. The role of radiation therapy in low-grade ependymomas is unclear.
METHODS: We report the results of 49 patients over an 18-year-period. Patients' clinical and radiologic data were analyzed retrospectively.
RESULTS: Mean age of patients at time of surgery was 46.4 years. There were 29 women (59%) and 20 men (41%). Gross total resection was achieved in 72% of patients. Tumors affected cervical spinal cord in 14 patients (29%), cauda equina and filum terminale in 10 (21%), conus medullaris in 8 (16%), thoracic spinal cord in 7 (14%), and cervicolumbar or thoracolumbar junction in 10 (20%). Postoperative radiation therapy was performed in 6 cases. Long-term follow-up was available for 39 patients (mean 50.7 months). High preoperative McCormick grade correlated with high risk for clinical deterioration postoperatively. On long-term follow-up, clinical status was the same as preoperatively in 51% of patients, worse in 21%, and improved in 28%. We found no differences in progression-free survival with regard to histology. Four tumors relapsed after progression-free survival of a mean of 21 months.
CONCLUSIONS: Gross total resection is considered the first choice in treatment of spinal ependymomas. The most important predictor of clinical outcome is preoperative neurologic functioning. The role of postoperative radiation needs further clarification.

Distinctive Surgical Strategy for Removal of String of Beadlike Schwannomas of Cauda Equina.

World Neurosurg. 2018 Mar;111:207-210

Authors: Liao D, Zhang J, Li D, Deng X, Ren Q, Chen H

Abstract
BACKGROUND: Spinal schwannomas are the most common intradural extramedullary tumors. However, a string of beadlike schwannomas is rare. In some cases, the beadlike tumors might present a multiple segmental growing pattern, often located in the lumbar spinal canal and on 1 nerve fiber. Despite its benign nature, the resection of this string of beadlike tumors could be a challenge to neurosurgeons, especially when the tumors extend to a long distance.
CASE DESCRIPTION: A 50-year-old female was admitted to our hospital, and her diagnosis was beadlike schwannomas. We performed 2 small hemilaminectomies and pulled all the tumors out gently. The patient made a full recovery quickly after resection.
CONCLUSIONS: In this case, we first reported a new surgical approach for the removal of a string of beadlike cauda equina schwannomas. We hypothesize that this procedure would be a potential addition to the present surgical methods under some circumstances.

Abstract
BACKGROUND: Ganglioglioma is a rare, benign, intraaxial glioneuronal tumor but a relatively common cause of pharmacoresistant temporal lobe epilepsy (TLE). Given its often nonspecific neuropsychiatric manifestations and frequently negative electroencephalographic workup, TLE can be easily misdiagnosed as a psychiatric disorder, particularly panic attacks.
CASE DESCRIPTION: We present a case of a 17-year-old boy who was found to have lesional TLE secondary to a left temporal ganglioglioma, 5 years after having been misdiagnosed with panic disorder and having undergone ineffective and unnecessary psychotherapy. He was successfully cured by surgery. Although a few similar cases of TLE masquerading as a panic disorder have been previously reported in the literature, this is the youngest and only pediatric patient described to date.
CONCLUSION: This report underscores the challenges in making an accurate clinical diagnosis of TLE and the importance of timely brain imaging whenever an atypical or medically refractory panic disorder is encountered.

Abstract
BACKGROUND: The recurrence rate of chronic subdural hematoma (CSDH) after trepanation is relatively high and involves various factors. We encountered an extremely rare case in which metastasis of an extracranial malignant tumor to the hematoma capsule was thought to be causing short-term repeated recurrences of CSDH.
CASE DESCRIPTION: The patient was a 74-year-old man who had undergone bur hole evacuation of left CSDH 7 months earlier and had been receiving chemotherapy for stage IV gastric cancer for the preceding 6 months. He presented with symptoms of right hemiparesis and was diagnosed with left CSDH. Bur hole evacuation was performed twice, but the hematoma enlarged again both times within a short period. A third bur hole evacuation was performed after middle meningeal artery embolization, but the hematoma again enlarged shortly thereafter. Hematoma enlargement was finally suppressed by extracting the hematoma capsule under craniotomy. In histopathologic examinations, hematoxylin-eosin staining showed poorly differentiated adenocarcinoma primarily along the luminal side of the hematoma capsule and immunohistochemical staining showed results identical to findings from the existing gastric cancer. Pathologic results confirmed metastasis of the gastric cancer to the hematoma capsule, and this was considered to be the cause of short-term repeated recurrence of CSDH.
CONCLUSIONS: To the best of our knowledge, this is the first report of metastasis to a hematoma capsule originating from an extracranial malignant tumor. The clinical course in the present case suggests metastasis of extracranial malignant tumor to the CSDH capsule as an extremely rare cause of recurrence.

Abstract
BACKGROUND: Brainstem cavernomas (BSCs) are rare and difficult to treat. The treatment strategy for symptomatic lesions in elderly patients remains unclear. In this review, we discuss the optimal treatment strategy and consider effective surgical strategies in elderly patients.
CASE DESCRIPTION: The clinical data of 8 elderly patients (age >70 years) with symptomatic BSCs drawn from the literature and 2 of our cases are summarized in this review. The mean patient age was 73.3 ± 3.13 years, and the most common location was the pons. Multiple rebleeding before surgery was seen in at least 4 cases, with clinical presentation and surgical approach varying depending on the location. Surgical removal was performed in 9 cases, and 1 case was treated with radiosurgery. The mean duration of clinical follow-up was 26.1 ± 18.2 months. Neurologic improvement was found in 5 cases, and postoperative decline was seen in 1 of the surgery cases.
CONCLUSIONS: Radical resection of the cavernoma with severe symptoms might be recommended in elderly patients, especially in those with multiple rebleeding events. From the viewpoint of surgery, we consider the subacute phase the optimal time to remove cavernomas in elderly hemorrhagic patients. However, multiple rebleeding events might exacerbate the neurologic deficit. Therefore, the subacute phase from the first or second rebleeding might be the best time for the surgical resection. At surgical intervention, preservation of the surrounding brain should be prioritized over complete removal of the cavernoma and hematoma.

Abstract
BACKGROUND: Cystic cerebral cavernomas belong to a rare subset of cavernous malformations in the brain. At present, there is limited information with regard to the demographics and etiology of this condition.
CASE DESCRIPTION: The authors present a case of a previously well 13-year-old male who presented with symptoms secondary to a cystic lesion of the fourth ventricle compressing on his brainstem. He underwent stereotactic aspiration of the cystic lesion and insertion of an Ommaya reservoir in the same setting. Postoperatively, his symptoms improved. The cyst fluid did not yield any positive cytology or culture results. A repeat MRI brain did not demonstrate evidence of any underlying lesion. However, the patient was readmitted for hemorrhage into the region of his previous fourth ventricular lesion. Surgical evacuation of the haematoma was performed and intraoperative tissue was sent for pathological interrogation. The final histology reported a cavernoma.
CONCLUSIONS: This is an unusual presentation of an intracranial vascular anomaly in a pediatric patient. Key features of the case include the diagnostic dilemma and management challenges faced from a neurosurgical perspective.

Abstract
BACKGROUND: Glioblastoma, also known as glioblastoma multiforme, is the most common primary malignant cerebral tumor in adults. Although glioblastoma multiforme is one of the most aggressive tumors in the brain, propagation through the dura mater is rare.
CASE DESCRIPTION: A 59-year-old man presented with progressive headache and aphasia. Magnetic resonance imaging identified an abnormal mass extending transcranially through the widened foramen rotundum into the infratemporal fossa and cavernous sinus. Emergency surgery was performed because of the patient's disturbed consciousness and uncal herniation. The pathologic diagnosis was glioblastoma with isocitrate dehydrogenase 1 (IDH-1) wild type arising in the left temporal region of the brain, penetrating the dura mater and propagating to the middle fossa with enlargement of the foramen rotundum. The tumor was resected, and radiochemotherapy with temozolomide was administered.
CONCLUSION: Although the mechanism of tumor spread is unknown, we hypothesized that originally there may have been spontaneous dural defects or thinning, such as a meningoencephalocele in the middle fossa, and the tumor coincidentally occurred there.

Abstract
BACKGROUND: Trastuzumab emtansine, an antibody-drug conjugate commonly abbreviated as T-DM1, is accepted as effective therapy for trastuzumab-resistant metastatic HER2-positive breast cancer. T-DM1 significantly increases progression-free and overall survival when compared with lapatinib plus capecitabine in patients with HER2-positive breast cancer previously treated with trastuzumab and a taxane. Among the common side effects related to T-DM1, thrombocytopenia and mucosal hemorrhage are seen, although they are infrequently judged to be clinically significant. Intracranial hemorrhages are extremely rare, and only 3 cases of hematomas have been reported in association with T-DM1 and remote radiotherapy, 2 of them with progressive enlargement.
OBJECTIVE: Herein we describe a patient who presented with a cerebellar hematoma that progressively enlarged over 8 months during treatment with T-DM1 and only a few months after whole-brain radiation therapy plus a stereotactic radiosurgery boost for a HER2-positive breast cancer cerebellar metastasis. The pathology of the hematoma was similar to that in previous cases and suggested a unique pathophysiology related to an interaction between T-DMI and radiation therapy.
CONCLUSIONS: A progressively enlarging intraparenchymal hematoma can be seen just a few months after delivery of radiation therapy for a metastatic brain lesion in HER2-positive breast cancer patients who are receiving T-DM1. In such patients, even a small focus of hemorrhage on magnetic resonance images should prompt close follow-up with serial imaging.

Abstract
A 23-year-old female was admitted to our hospital because she had suffered from back pain for 3 years and paralysis of both lower limbs for 10 days. Neurologic examination showed sensory disturbance and complete paralysis in bilateral lower extremities with negative Babinski sign. Contrast-enhanced magnetic resonance imaging showed there was an enhanced intradural lesion between T2 and T12, which pressed the spinal cord. The lesion was resected completely by laminectomy approach, and the tumor was totally intradural extramedullary. Postoperative pathologic findings confirmed that the lesion was ependymoma. Preoperative symptoms recovered slightly after surgery. The back pain disappeared and muscle strength of both lower extremities returned to level 4 one year after the operation. Meanwhile, her sensory function in both legs improved but was not completely normal. Intradural extramedullary primary ependymomas are rare. To our knowledge, this case is the largest one ever reported in the literature.

Small Cell Glioblastoma of the Sella Turcica Region: Case Report and Review of the Literature.

World Neurosurg. 2018 Feb;110:174-179

Authors: Deng S, Liu L, Wang D, Tong D, Zhao G

Abstract
BACKGROUND: Glioblastomas in the sellar region are very rare; in most cases, the tumor originates from the optic nerve or optic chiasm. Only 4 cases of sellar glioblastoma with a nonoptic origin have been reported. We present such a case with detailed clinical, imaging, and histopathologic information. We also review similar published cases.
CASE DESCRIPTION: A 42-year-old woman presented with endocrinologic abnormalities, including amenorrhea and lactation, symptoms of diabetes insipidus, and signs of elevated intracranial pressure. Magnetic resonance imaging showed a giant, heterogeneously enhancing lesion involving the intrasellar, parasellar, and suprasellar regions, with hypercellularity and signs of infiltration of adjacent structures. Intraoperative examination revealed the tumor to be independent from the optic pathways, but it showed infiltration of the hypothalamic region. Histopathologic examination demonstrated uniformly packed small cells and negative staining for glial fibrillary acidic protein, which was consistent with a diagnosis of small cell glioblastoma.
CONCLUSIONS: This is the first report of a small cell glioblastoma in the sella turcica region. Glioblastomas in the sellar region with no clear evidence of an optic origin should be viewed as an independent disease entity. The typical characteristics of this tumor indicate it should be considered a rare subtype of glioblastoma. Further accumulation of experience is needed to better differentiate these cases and to offer optimal treatment.

Abstract
BACKGROUND: Abscess coexisting within a brain tumor is rare. Case reports in the literature primarily consist of sellar pathology and parenchymal lesions, including meningioma, glioma, and metastases. We report a case of glioblastoma with an intratumoral abscess in a middle-aged patient with no prior invasive procedure or systemic focus of infection.
CASE DESCRIPTION: A 45-year-old woman presented with new-onset generalized seizures and rapidly progressive left hemiparesis. Imaging showed a right frontal necrotic lesion with a peripherally enhancing wall and solid component posteriorly. There was no diffusion restriction within the lesion. She was afebrile, and there was no systemic focus of infection. With provisional diagnosis of malignant glioma, she underwent surgical resection of the lesion. A differential diagnosis of abscess was considered preoperatively because of the rapid increase in size of the lesion. At surgery, there was a pus-filled cavity with a few areas of grayish, soft, flimsy wall and thrombosed veins. This raised a strong suspicion of a coexisting abscess within a malignant glioma, which was confirmed by histopathologic and microbiologic examination.
CONCLUSIONS: It is important for neurosurgeons to be aware of this rare entity. The treatment protocol remains controversial and is primarily guided by expert opinion. It is important to aggressively treat the patient with antibiotics followed by adjuvant therapy for malignancy. The timing and administration of adjuvant therapy are unclear. We suggest a delay of chemotherapy until at least 4 weeks of therapy with sensitive antibiotics.

Abstract
BACKGROUND: Chordomas are rare tumors showing locally aggressive nature and high rates of local recurrences. Distant metastases are infrequently reported and cardiac metastases are extremely rare.
CASE DESCRIPTION: A 32-year-old woman had been diagnosed with a large chordoma involving the clivus. She received a total removal of the tumor and adjuvant intensity-modulated radiotherapy. Four and a half years after the surgery, she presented with multifocal lesions in the ventricles and the right atrium, although an open thoracotomy revealed chronic inflammation. Two years after finding the cardiac lesions, thoracoscopic biopsy of the chest wall lesion revealed metastatic chordoma. She received palliative imatinib for 2 weeks before she died of tumor progression.
CONCLUSION: This case illustrates the metastatic potential of clivus chordoma, and the heart can be a site of metastasis.

Abstract
OBJECTIVE: Cortical ependymomas (CEs), supratentorial ependymomas that selectively involve the cerebral cortex, are relatively rare neoplasms that have not been extensively described. The purpose of our study was to identify the clinical features, radiologic characteristics, and treatment of a series of such tumors.
METHODS: Thirteen patients with CEs from our hospital were included in this study. Epidemiologic characteristics, clinical features, imaging findings, treatment methods, and clinical outcomes were reviewed retrospectively.
RESULTS: The patients consisted of 7 men and 6 women with mean age of 31.1 ± 23.2 years (range, 4-74 years). The most common clinical manifestation was seizure (n = 11; 85%), followed by headache (n = 2; 15%). None of the tumors were incidentally detected. Eight CEs were located in the right hemisphere and 5 in the left side. The 2 most common tumor locations were the frontal (n = 5; 38%) and parietal lobe (n = 5; 38%). All patients underwent surgical resection. Gross total resection was achieved in 12 patients (92%), and subtotal resection was performed in 1 patient (8%). Ten of the 11 patients who presented with seizure are seizure-free after surgery (91% seizure-free rate). According to the World Health Organization classification system, 9 tumors (69%) were Grade II (ependymoma) and 4 (31%) were Grade III (anaplastic ependymoma). The mean follow-up was 52 months (range, 20-88 months). No recurrence was observed in patients with Grade II CEs. Of 4 patients with Grade III CEs, 2 (50%) suffered from tumor recurrence after initial treatment.
CONCLUSIONS: CEs are a rare subset of supratentorial ependymomas that selectively involve the cerebral cortex. Most CEs are low grade and present with seizures. Anaplastic CEs show a greater recurrence rate and a relatively poor prognosis. Gross total resection with or without adjuvant radiotherapy is currently the optimal treatment for CEs. CEs seem to have a more favorable prognosis than other supratentorial ependymomas.

Dural-Based Cavernous Malformation at the Cerebral Convexity: Report of Two Pediatric Patients.

World Neurosurg. 2018 Apr;112:81-85

Authors: Li G, Zhai X, Zhang Y, Liang P, Wu X, Hou K

Abstract
BACKGROUND: Intracranial cavernous malformations (CMs) are usually located at the cerebral parenchyma; dural-based CMs outside the middle fossa are rarely reported. To our knowledge, dural-based CMs located at the cerebral convexity are even rarer in that only 2 pediatric cases have ever been reported. In this report, we present 2 extremely rare cases of dural-based CMs at the cerebral convexity in pediatric patients. The clinical course, radiologic and pathologic features, treatment, and follow-up are described.
CASE DESCRIPTION: The first case is a 6-year-old boy who presented with headache and vomiting and was found to have an acute subdural hematoma and space-occupying lesion. Intraoperative findings and histologic examination were consistent with a CM. He experienced an uneventful postoperative recovery. The second case is a 43-day-old female neonate who presented with a progressively enlarging neoplasm at the right occipital region since birth. Computed tomography of the head performed at admission showed a slight hyperdense occupying lesion communicating between the intra- and extracranial cavity through a skull defect. The lesion was resected en bloc and histologic examination was in accord with a CM.
CONCLUSIONS: The clinical manifestations and radiologic characteristics of dural-based CMs are nonspecific. Unlike that of their cerebral parenchymal counterparts, the radiologic appearance of dural-based CMs is confusing and misleading. Surgical resection is the primary treatment selection for dural-based CMs. In cases with no close relationship to dural sinuses, complete surgical resection with minimal blood loss and few neurologic deficits could be easily achieved.

Abstract
BACKGROUND: Spinal intradural arachnoid cysts are rare in the pediatric population. We present a rare case of intradural spinal arachnoid cysts that spontaneously and repeatedly disappeared and reoccurred.
CASE DESCRIPTION: A 2-year-and-8-months-old boy presenting with lower extremity weakness was found to have spinal intradural arachnoid cysts in cervical and thoracolumbar regions at separate times. Although spontaneous disappearance of both lesions was observed, surgical treatment was finally performed for the symptomatic recurrent thoracolumbar lesion.
CONCLUSIONS: To the best of our knowledge, this is the first report of spontaneously disappearing and recurring spinal arachnoid cysts.

Abstract
BACKGROUND: We report a rare case of hemorrhagic cavernous sinus hemangioma with sudden onset of abducens palsy.
CASE DESCRIPTIONS: Sudden onset of diplopia occurred in a 49-year-old female. Head computed tomography findings revealed a high-density mass in the right cavernous sinus with protrusion to the sphenoid sinus. Endoscopic endonasal surgery was performed, though early recurrence was noted. A Gamma Knife surgical procedure was then performed to prevent early recurrence after histopathologic confirmation of cavernous sinus hemangioma. Thereafter, the tumor was controlled and abducens palsy disappeared.
CONCLUSIONS: For treatment of cavernous sinus hemangioma, stereotactic radiosurgery should be considered as primary or adjuvant treatment in the very early phase after removal, to avoid early recurrence.

Abstract
BACKGROUND: Liponeurocytoma is a very rare tumor classified as grade II (neuronal and mixed neuronal-glial tumors) according to 2016 World Health Organization classification of tumors of the central nervous system. The median age at detection is 50 years, and the most frequent location is the posterior cranial fossa, especially within the cerebellar hemispheres; liponeurocytomas arising in the cerebellopontine angle (CPA) are exceptional.
CASE DESCRIPTION: Here we report the clinical, radiological, and pathological characteristics of a CPA liponeurocytoma in a 35-year-old woman, as well as a review of the literature. This unusual cisternal location raises the issue of the differential imaging diagnosis with much more common CPA tumors (e.g., meningiomas, vestibular schwannomas, ependymomas, epidermoid cyst, hemangioblastomas, medulloblastomas).
CONCLUSION: To the best of our knowledge, 59 cases of cerebellar liponeurocytomas have been reported to date, which include only 6 cases of CPA liponeurocytomas. Treatment relies on total removal whenever possible, with an excellent prognosis, but a high MIB-1 index (>10%) and/or incomplete tumor resection are the main adverse prognostic factors.

Abstract
BACKGROUND: Pleomorphic xanthoastrocytomas are rare tumors of glial origin comprising <1% of all astrocytic tumors of brain. These tumors are rare in the infratentorial compartment and were not known to arise from the middle cerebellar peduncle.
CASE DESCRIPTION: We discuss the case of a 16-year-boy who presented with altered sensorium and features of increased intracranial pressure and was found to have a 4 × 4 × 3 cm mass arising from the middle cerebellar peduncle.
CONCLUSION: We also discuss the histopathologic features of these tumors suggesting their resemblance to other maldevelopmental tumors, the rarity of pure World Health Organization grade II pleomorphic xanthoastrocytoma in the infratentorial compartment, as well as the appropriate management.

Spinal Epidural Cavernous Hemangioma: A Clinical Series of 7 Patients.

World Neurosurg. 2018 Mar;111:e183-e191

Authors: Gao C, Xu W, Qing Lian J, Feng D, Cui JF, Liu H

Abstract
OBJECTIVE: To investigate imaging characteristics of spinal epidural cavernous hemangiomas (ECHs) and improve understanding of this rare disease.
METHODS: This retrospective study included 7 cases of ECH, confirmed with surgical pathology and imaging. All patients underwent computed tomography and conventional magnetic resonance imaging. One patient underwent diffusion-weighted imaging.
RESULTS: ECH originated from vertebrae with coarsened trabeculae in 5 patients. Among these, 1 tumor was mainly in the intervertebral foramen and paravertebral space, 2 extended to the intraspinal and paravertebral spaces and appeared dumbbell-shaped, and 2 were located in the intraspinal space. Purely epidural hemangiomas were present in 2 patients; 1 of these patients had adjacent bony erosion. Epidural hemangiomas were isointense in 6 patients and hyperintense in 1 patient on T1-weighted images; lesions were hyperintense on T2-weighted images in all 7 patients. Peripheral fat was present in 4 ECHs; 2 were of vertebral origin, and 2 were of epidural space origin. In the patient who underwent diffusion-weighted imaging, the lesion showed restricted diffusion. The affected vertebra had coarsened trabeculae, which may be a critical finding for differentiating ECHs of vertebral origin from foraminal nerve sheath tumors. Peripheral fat around hemangiomas of vertebral origin and primary epidural space hemangiomas indicates chronicity.
CONCLUSIONS: ECHs are classified either as epidural hemangiomas of vertebral origin or as primary epidural hemangiomas. Awareness of imaging characteristics of ECHs may facilitate diagnosis and treatment of these lesions.

Abstract
Cavernous sinus hemangiomas (CSHs) are rare vascular tumors, which are infrequently encountered in everyday neurosurgical practice. Attempted resection of this lesion without preoperative planning carries a high risk for significant blood loss and cranial neuropathies. Radiation treatments can provide an alternative and safe treatment modality for these lesions. Therefore an increased awareness is warranted for their clinical suspicion. We reported on a patient who presented with visual deficits and a mass in the sellar, suprasellar, and middle fossa regions. Given the high suspicion for a CSH due to characteristic radiologic findings, a keyhole craniotomy approach was safely used for an open biopsy. The final pathology was consistent with a CSH, and the patient subsequently underwent radiation therapy without complications.

Abstract
OBJECTIVE: The aim of this study was to perform an integrated survival analysis of patients with bilateral thalamic glioma and to assess the influence of various prognostic factors on overall survival.
METHODS: A literature search of PubMed, Web of Science, and Google Scholar was performed for literature in English published from 1964 to May 2017. Detailed information including demographics, clinical characteristics, treatments, critical events, and time to events for survival analysis were extracted from the included articles. In addition, 2 cases diagnosed in our institution were included.
RESULTS: The study included 53 cases from 32 published articles and 2 cases from our institution that were selected for analysis. Univariate analysis showed the duration of symptoms (≥2 or <2 months), glioma type (astrocytoma or glioblastoma multiforme), and World Health Organization (WHO) grade (low or high) had a significant correlation with overall survival (log-rank P = 0.011, 0.001, and <0.001, respectively). Multivariate analysis showed that the duration of symptoms (hazard ratio [HR], 0.299; 95% confidence interval [CI], 0.121-0.736; P = 0.009), and WHO grade (HR, 4.639; 95% CI, 1.891-11.382; P = 0.001) were independent prognostic factors for bilateral thalamic glioma (BTG) survival.
CONCLUSIONS: This comprehensive analysis of rare BTG patients revealed that a longer duration of symptoms (≥2 months) and low WHO grade were significantly associated with improved survival and were independent prognostic factors for overall survival.

Meningioma with the unique coexistence of secretory and lipomatous components: A case report with immunohistochemical study.

Indian J Pathol Microbiol. 2017 Jul-Sep;60(3):381-384

Authors: Patil PR, Warpe BM, Juvekar VH, Manohar V

Abstract
Meningioma is the most common extra-axial neoplasm which accounts for 30% of all intracranial tumors with a female predilection. These tumors exhibit a broad spectrum of differentiation potency corresponding to different histological subtypes. Meningothelial cells can rarely show separate secretory or lipomatous (metaplastic) transformation. The coexistence of these two different histological subtypes in a single meningeal tumor is extremely rare with only two reported cases. We report an uncommon case of meningioma in a 40-year-old female showing histological admixture of both secretory and lipomatous (metaplastic) meningioma, which to our knowledge is only the third reported case in the world. It highlights the multipotency of phenotypic transformation of primary meningothelial cells. This rare tumor behaves in a fashion similar to benign meningioma of WHO Grade I type.

Abstract
OBJECTIVE: Primary central nervous system lymphoma (PCNSL) is a specific type of non-Hodgkin lymphoma with poor prognosis. The rare incidence of this disease and difficulty to obtain sufficient tissue material impede deep research into PCNSL. However, application of modern molecular techniques makes it possible to find biological characteristics exclusive to PCNSL. Therefore, we systematically reviewed the latest research progress on biological characteristics and pathogenesis of PCNSL.
DATA SOURCES: The data analyzed in this review were from the articles listed in PubMed database.
STUDY SELECTION: Articles focusing on the biology of PCNSL at the cytogenetic or molecular level were reviewed, including clinical, basic research, and review articles.
RESULTS: With respect to histopathology, perivascular growth pattern and reactive perivascular T-cell infiltration are regarded as typical histopathological manifestations of tumor cells in PCNSL. Moreover, tumor cells of PCNSL predominantly express an activated B-cell-like phenotype, including CD10- BCL-6+ MUM1+, CD10- BCL-6- MUM1+, and CD10- BCL-6- MUM1-. On the molecular level, some molecular and genetic alterations may contribute to malignant transformation, including mutations of proto-oncogenes and tumor suppressor genes, gains and losses of genetic material, as well as aberrant activation of some important signaling pathways, such as nuclear factor-κB and JAK/STAT pathway.
CONCLUSIONS: The integrated molecular mechanisms involved in pathogenesis of PCNSL are not well understood. The important biomarkers indicating prognosis are not identified. Multicenter studies should be carried out to elucidate pathogenesis of PCNSL to find novel and effective therapeutic strategies.

Abstract
INTRODUCTION: Malignant primary diffuse leptomeningeal gliomatosis (MPDLG) are rare central nervous system neoplasms associated with a poor outcome.
CASE REPORT: We report the case of a 40-year-old woman who presented with unusual worsening of bilateral sciatica, headaches, diplopia and a left proptosis. MRI of the head and spine showed multiple leptomeningeal lesions with no intra parenchymal involvement. The search for a primary tumor was negative. An open surgical biopsy of the prominent intradural lumbar tumor was performed within a week. Histopathology, immunochemistry and molecular analyses revealed a malignant glioma with histone H3.3 K27M mutation. The patient was referred to the neuro-oncologist for chemotherapy and craniospinal radiotherapy. Despite aggressive therapy, she died of disseminated tumoral progression, 18 weeks after the diagnosis.
CONCLUSION: MPLG is a rare tumor which should be considered whenever a patient presents with diffuse or multinodular meningeal contrast-enhancing lesions. Some cases of MLPG share histological and immunophenotypical features with diffuse midline gliomas H3-K27M-mutant, a rapidly fatal disease. The diagnosis remains histopathological and, therefore a biopsy is obligatory without delay. Immunohistochemistry and/or molecular analyses are now currently essential for a formal classification and, to provide a better prediction of clinical outcome, particularly in this heterogeneous group of tumors.

Abstract
BACKGROUND: Pituitary ependymoma is exceptionally rare. Its etiology, clinical presentation, radiologic feature, and treatment strategy are still a matter of debate. Only 7 human cases with limited data were reported in the English literature, and now we described another case of pituitary ependymoma. We also systematically reviewed previously reported cases and described its potential etiology, clinical presentation, radiologic features, pathology, immunohistochemical analysis, and ultrastructural examinations.
CASE DESCRIPTION: A lesion in pituitary fossa was discovered in a 40-year-old man after suffering a progressive deterioration of vision in his right eye for >1 year with intermittent headache. The lesion was microsurgically resected and proved to be ependymoma upon pathologic and histologic examination. The patient made a fully recovery after surgery.
CONCLUSIONS: To our knowledge, only 7 patients with ependymoma in the sellar region have been described in the English literature. We reported 1 more case of pituitary ependymoma and discussed the potential etiology, clinical presentation, radiologic features, pathology, immunohistochemical analysis, ultrastructural examinations, treatment, surgery, radiotherapy, chemotherapy, and prognosis of pituitary ependymoma. The case report may serve as a helpful reference for clinicians and radiologists.

Childhood cancer incidence by ethnic group in England, 2001-2007: a descriptive epidemiological study.

BMC Cancer. 2017 Aug 25;17(1):570

Authors: Sayeed S, Barnes I, Ali R

Abstract
BACKGROUND: After the first year of life, cancers are the commonest cause of death in children. Incidence rates vary between ethnic groups, and recent advances in data linkage allow for a more accurate estimation of these variations. Identifying such differences may help identify potential risk or protective factors for certain childhood cancers. This study thus aims to ascertain whether such differences do indeed exist using nationwide data across seven years, as have previously been described in adult cancers.
METHODS: We obtained data for all cancer registrations for children (aged 0-14) in England from January 2001 to December 2007. Ethnicity (self-assigned) was established through record linkage to the Hospital Episodes Statistics database or cancer registry data. Cancers were classified morphologically according to the International Classification of Childhood Cancer into four groups - leukaemias; lymphomas; central nervous system; and other solid tumours. Age standardised incidence rates were estimated for each ethnic group, as well as incidence rate ratios comparing each individual ethnic group (Indian, Pakistani, Bangladeshi, Black African, Black Carribean, Chinese) to Whites, adjusting for sex, age and deprivation.
RESULTS: The majority of children in the study are UK born. Black children (RR = 1.18, 99% CI: 1.01-1.39), and amongst South Asians, Pakistani children (RR = 1.19, 99% CI: 1.02-1.39) appear to have an increased risk of all cancers. There is an increased risk of leukaemia in South Asians (RR = 1.31, 99% CI: 1.08-1.58), and of lymphoma in Black (RR = 1.72, 99% CI: 1.13-2.63) and South Asian children (RR = 1.51, 99% CI: 1.10-2.06). South Asians appear to have a decreased risk of CNS cancers (RR = 0.71, 99% CI: 0.54-0.95).
CONCLUSIONS: In the tradition of past migrant studies, such descriptive studies within ethnic minority groups permit a better understanding of disease incidence within the population, but also allow for the generation of hypotheses to begin to understand why such differences might exist. Though a major cause of mortality in this age group, childhood cancer remains a relatively rare disease; however, the methods used here have permitted the first nationwide estimation of childhood cancer by individual ethnic group.

Strategy for patients with co-existence of meningioma and intracerebral aneurysm, especially unruptured aneurysm (-seven cases and review of the literature-).

J Clin Neurosci. 2017 Nov;45:236-242

Authors: Takeda N, Nishihara M, Yamanishi S, Kidoguchi K, Hashimoto K

Abstract
BACKGROUND: Intracerebral aneurysms co-existing with meningiomas are rare. Treatment strategies for intracerebral aneurysms co-existing with meningiomas have not yet been established.
METHODS: We studied 62 patients with intracerebral aneurysms co-existing with meningiomas in the literature including our seven cases, evaluated the various managements and outcomes, and discussed the strategy for intracerebral aneurysms, especially unruptured cases, co-existing with meningiomas. The aim of this study was to develop a guide for the management of non-subarachnoid hemorrhage (SAH) intracerebral aneurysms co-existing with meningiomas.
RESULTS: Most intracerebral aneurysms co-existing with meningiomas are unruptured. Of course, aneurysms presenting with SAH should be treated first followed by the resection of meningiomas. In addition, intracerebral aneurysms inside or adjacent to meningiomas have a high risk of intraoperative rupture during the surgery for meningiomas, and it may be necessary to treat them first followed by the resection of meningiomas with one or two-step surgery. In nine out of 62 patients, ten intracerebral unruptured aneurysms were not treated; however, no intracerebral aneurysms ruptured during the follow-up period, and outcomes of these patients were good in eight and poor in only one.
CONCLUSIONS: Intracerebral unruptured aneurysms remote from meningiomas may be treated according to the guidelines for unruptured aneurysms. In advance of microsurgery and endovascular techniques, both lesions should be treated, if possible.

Abstract
Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is a rare multisystemic autosomal dominant disorder typically presenting with cutaneous basal cell carcinomas, multiple keratocysts, and skeletal anomalies. NBCCS is caused by heterozygous mutations in the PTCH1 gene in chromosome 9q22, in the PTCH2 gene in 1p34, or the SUFU gene in 10q24.32. Here, we report on an 18-month-old boy presenting with medulloblastoma, frontal bossing, and multiple skeletal anomalies and his father who has basal cell carcinomas, palmar pits, macrocephaly, bifid ribs, calcification of falx cerebri, and a history of surgery for odontogenic keratocyst. These clinical findings were compatible with the diagnosis of NBCCS, and a novel mutation, c.1249delC; p.Gln417Lysfs*15, was found in PTCH1 causing a premature stop codon.

Abstract
BACKGROUND: Posterior cranial fossa (PCF) hemangioblastomas are benign, highly vascularized, and well-differentiated tumors with well-described histopathologic features. Although relatively rare, this tumor is the most prevalent primary tumor of the cerebellum in adults.
OBJECTIVE: Because the demographics of patients with such a tumor (as well as the clinical, morphologic, pathologic, surgical features, and outcomes) are not fully understood, we systematized characteristic patient and tumor features.
METHODS: We undertook a systematic review of the English-language literature in PubMed for PCF hemangioblastomas in adults published in the past 31 years. We analyzed geographic distribution and year of publication of articles; demographic data of patients; presenting symptoms and clinical signs; tumor location and morphology; histopathologic features, extent of tumor resection, perioperative blood loss, and postoperative complications; length of hospital stay; and outcomes.
RESULTS: We reviewed 207 articles describing 1759 infratentorial hemangioblastomas in a cohort of 1515 adult patients. We found female predominance in patients with Von Hippel-Lindau disease (VHLD) compared with male predominance in the general patient group. Symptoms of intracranial hypertension were more common in the VHLD group compared with the general group of patients. The cerebellar location was more common in the VHLD group and solid (parenchymatous) tumor was the most common type. Most patients underwent total resection but rate of resection did not differ between the general and VHLD groups. Most patients had a favorable outcome.
CONCLUSIONS: The literature of adult PCF hemangioblastomas is limited and general surgical experience with such tumors is scarce because of their rarity. Rates of postoperative complications and mortality remain higher than expected. However, prognosis and surgical outcomes are generally favorable. Nevertheless, surgery of adult PCF hemangioblastomas is a demanding and challenging task.

Primary Central Nervous System T-Cell Lymphoma With Aberrant Expression of CD20 and CD79a: A Diagnostic Pitfall.

Int J Surg Pathol. 2017 Oct;25(7):599-603

Authors: Gupta N, Nasim M, Spitzer SG, Zhang X

Abstract
Primary central nervous system T-cell lymphoma (PCNSTCL) is rare, accounting for 2% of CNS lymphomas. We report the first case of PCNSTCL with aberrant expression of CD20 and CD79a in an 81-year-old man with a left periventricular brain mass. A biopsy revealed dense lymphoid infiltrate consisting of medium-sized cells in a background of gliosis and many histiocytes. The lymphoid cells were positive for CD2, CD3, CD7, CD8, T-cell intracellular antigen-1, granzyme B, CD20, and CD79a and negative for CD4, CD5, PAX-5, OCT-2, BOB-1, human herpes virus-8, and Epstein-Barr virus-encoded small RNAs. Molecular studies revealed clonal TCR-β and TCR-γ gene rearrangements and negative immunoglobulin gene rearrangements. The patient was treated with chemotherapy (vincristine and methotrexate) and rituximab, but he died 1 month after the diagnosis. This is a unique case that emphasizes the use of a multimodal approach, including a broad immunohistochemical panel and molecular studies in lineage determination for lymphomas with ambiguous phenotype.

Abstract
Malignancies arising from the central nervous system are rare. Brain metastases, in contrast, are perhaps the most common neurologic complication of cancer. Radiotherapy, as part of combined modality therapy, continues to evolve with the advancement of stereotactic radiosurgery indications, the addition of new technologies, such as alternating electric field therapy, and mounting advances in the complex biology of these entities. The explosion of new clinical trials combined with newly discovered molecular markers suggest the beginning of a paradigm shift in the management of these challenging malignancies that will allow for future risk-stratification strategies.

Anaplastic Ependymoma With Ganglionic Differentiation: Report of a Rare Case and Implications in Diagnosis.

Int J Surg Pathol. 2017 Oct;25(7):644-647

Authors: Rao S, Ravindra N, Sadashiva N, Indira Devi B, Santosh V

Abstract
Ependymomas are glial neoplasms with rare cases exhibiting neuronal differentiation. We describe a case of spinal anaplastic ependymoma with ganglionic differentiation in a 28-year-old woman. The ganglionic component was labeled by synaptophysin, whereas the rest of the tumor showed features of an anaplastic ependymoma. Stem cell marker MELK was noted to stain both the neoplastic ependymal and ganglionic components, possibly suggesting a stem cell/progenitor origin for the tumor with subsequent divergent differentiation.

Abstract
Background: Primary extranodal marginal zone lymphoma (MZL) of the dura is a rare neoplastic entity in the central nervous system (CNS).
Methods: We used literature searches to identify previously reported cases of primary dural MZL. We also reviewed clinical, pathologic, and radiographic data of an adult patient with concurrent dural MZL and chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL).
Results: We identified 104 cases of dural MZL in the literature. None of them presented concurrently with another type of non-Hodgkin lymphoma. This is the first report of composite lymphoma consisting of dural MZL and CLL/SLL in the bone marrow and lymph nodes.
Conclusion: Primary dural MZL is a rare, indolent low-grade CNS lymphoma, with a relatively good prognosis. Its treatment is multidisciplinary and often requires surgical intervention due to brain compression, along with low to moderate doses of radiotherapy and/or systemic chemotherapy.

Abstract
Accelerated atherosclerosis of intra- or extracranial large arteries is a major cause of radiation-induced strokes. Recent development of MRI techniques has enabled detection of another important vasculopathy: microinfarcts or microbleeds after cranial irradiation. The purpose of this study was to investigate the incidence and characteristics of small vessel disease after cranial irradiation. MR images and clinical records of 102 outpatients who had undergone cranial irradiation to brain tumors were retrospectively reviewed. Only those who had undergone T1WI, T2WI, FLAIR, and T2∗WI during follow-up were included. Small vessel disease including small subcortical infracts, microbleeds, and volume of white matter lesions were assessed on the latest MR images of each patient. Ischemic or hemorrhagic stroke during the follow-up period was also reviewed. Twelve patients (mean age at the latest MRI, 38.5years; 8men) were assessed. The total radiation dose was 52.3±9.7Gy in these patients, 9 of whom received whole brain irradiation. Small subcortical infarcts (mean 2.3) were detected in 8 patients, microbleeds (mean 19.4) were detected in 11, and white matter lesion volume was 38.3±11.6ml. During the follow-up period of 19.8±9.7years, 5 patients experienced stroke (4 lacunar, 1 hemorrhagic). These strokes occurred as long as a median 21.9 (range, 10.4-30.2) years after cranial irradiation. In conclusion, small vessel disease is not a rare complication after cranial irradiation, even in young patients. Patients after cranial irradiation should be followed up with MR imaging including a hemosiderin-sensitive sequence.

Waardenburg syndrome: a rare cause of inherited neuropathy due to SOX10 mutation.

J Peripher Nerv Syst. 2017 Sep;22(3):219-223

Authors: Bogdanova-Mihaylova P, Alexander MD, Murphy RPJ, Murphy SM

Abstract
Waardenburg syndrome (WS) is a rare disorder comprising sensorineural deafness and pigmentation abnormalities. Four distinct subtypes are defined based on the presence or absence of additional symptoms. Mutations in six genes have been described in WS. SOX10 mutations are usually associated with a more severe phenotype of WS with peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, and Hirschsprung disease. Here we report a 32-year-old man with a novel heterozygous missense variant in SOX10 gene, who presented with congenital deafness, Hirschsprung disease, iris heterochromia, foot deformity, and intermediate conduction velocity length-dependent sensorimotor neuropathy. This case highlights that the presence of other non-neuropathic features in a patient with presumed hereditary neuropathy should alert the clinician to possible atypical rare causes.

Granular cell tumour of the neurohypophysis: an unusual cause of hypopituitarism.

Endocrinol Diabetes Metab Case Rep. 2018;2018:

Authors: Bello CT, Cipriano P, Henriques V, Duarte JS, Marques CC

Abstract
Granular cell tumours (GCT) are rare, slow-growing, benign neoplasms that are usually located in the head and neck. They are more frequent in the female gender and typically have an asymptomatic clinical course, being diagnosed only at autopsy. Symptomatic GCT of the neurohypophysis are exceedingly rare, being less than 70 cases described so far. The authors report on a case of a 28-year-old male that presented to the Endocrinology clinic with clinical and biochemical evidence of hypogonadism. He also reported minor headaches without any major visual symptoms. Further laboratory tests confirmed hypopituitarism (hypogonadotrophic hypogonadism, central hypothyroidism and hypocortisolism) and central nervous system imaging revealed a pituitary macroadenoma. The patient underwent transcranial pituitary adenoma resection and the pathology report described a GCT of the neurohypophysis with low mitotic index. The reported case is noteworthy for the rarity of the clinicopathological entity.
Learning points: Symptomatic GCTs are rare CNS tumours whose cell of origin is not well defined that usually give rise to visual symptoms, headache and endocrine dysfunction.Imaging is quite unspecific and diagnosis is difficult to establish preoperatively.Surgical excision is challenging due to lesion's high vascularity and propensity to adhere to adjacent structures.The reported case is noteworthy for the rarity of the clinicopathological entity.

Abstract
Contrast reagents for ultrasound imaging are widely used in clinical medical diagnosis because ultrasound resolution is limited. Contrast agents must be utilized to enhance the image resolution. At present, microbubbles (MBs) and nanobubbles (NBs) are the main contrast reagent candidates for improving the signal resolution. Fluorescence upconversion nanoparticles provide high sensitivity and also function as nanocarriers. This can label tumor cells in a specific organ under irradiation of near-infrared (NIR) light. However, despite the use of NIR light, the penetration depth of NIR is only approximately 15 mm. Thus, we combine fluorescence with ultrasonic imaging to achieve the effect of multiple imaging and solve the low penetration depth of fluorescence imaging and the poor resolution of ultrasound imaging. The dual imaging modalities achieved higher resolution or signal to noise ratios. In this study, Nd3+-sensitized upconversion nanoparticles (UCNPs) are combined with graphitic carbon nitride quantum dots (CNs) and embedded in NBs (UCNP-CN@NBs). The UCNPs are excited by 808 nm light and emit visible and ultraviolet light. Then, the energy of the ultraviolet light is transferred to the CNs to produce reactive oxygen species (ROS) for photodynamic therapy. Ultrasonic waves are also used to promote NB bursting and the release of ROS molecules in photodynamic therapy, leading to cancer cell apoptosis.

Abstract
RATIONALE: Oligodendrogliomas are usually located in the frontal, parietal and the temporal lobe, with the ones in the fourth ventricle quite rare. Hence we want to introduce a case about the rare disease.
PATIENT CONCERNS: An eight-year old boy complained of progressive headache, dizziness and vomit for about 2 months. Then the slight ataxia was found by the physical examinations, with no sensory disturbances and other motor disturbances.
DIAGNOSES: Abnormal signals on the fourth ventricle were found by the preoperative brain computed tomography (CT) scan and magnetic resonance imaging (MRI) scan. So the patient accepted a gross total resection of the lesion with pathologically confirmed oligodendroglioma.
INTERVENTIONS: Radiotherapy was then delivered in 27 fractions at 2Gy per fraction after the operation, with one fraction daily for five days weekly. No other therapies were used for the patient.
OUTCOMES: The brain MRI was used for follow-up every three months until now when he has finished all therapies for more than one year. No progressive behaviors (for example, headache, dizziness, vomit and other symptoms about cerebellar tonsillar hernia) or images have been presented. And the follow-up will be continued.
LESSONS: Although oligodendrogliomas are usually located in the frontal lobe, with the ones of fourth ventricle extremely rare, they must be kept in mind all times. Treatments applied to our case may be provided as a reference for clinicians. Furthermore, the maximal range of resection, histologically proved oligodendroglioma and the 1p/19q loss of heterozygosity are associated with favorable prognosis.

Abstract
We report a rare long-surviving case of solitary brain metastasis from colon cancer. The patient was a 64-year-old female. She had undergone laparoscopic right hemicolectomy for ascending colon cancer in October 2012(pStage III b). She was discharged from the hospitalin a satisfactory state, but 26 days after the operation, she was transported to the emergency department for systemic tonic-clonic convulsions. Magnetic resonance imaging revealed solitary metastatic tumor in the brain, but there were no metastatic lesions in other organs. Metastatic lesion was small and complete removal was considered to be difficult. Thus, we planned stereotactic radiosurgery for the metastases, and then regimen of mFOLFOX6 plus Bmab, tegafur-uracil plus Leucovorin were administered. As a means of evaluating recurrence of brain metastasis, methionine positron emission tomography(methionine PET)was also performed, and the patient has survived for 54 months since the surgery, to date, without recurrence. Cases with brain metastasis of colorectal cancer often have metastases involving other organs and are considered to have a poor prognosis. For solitary brain metastasis, long-term survival can be obtained with multidisciplinary treatments, and methionine PET is considered to be usefulfor recurrence evaluation after radiation treatment.

Abstract
BACKGROUND: Ectopic pituitary adenomas (EPAs) are rare, and the suprasellar cistern seems to be the most common location. At this time, no detailed original classification, diagnosis, or treatment protocols for suprasellar pituitary adenomas (SPAs) have been described.
CASE DESCRIPTION: A 19-year-old man showed visual disturbances and lack of libido for 3 years, he suffered a sharp decline in vision with only light perception in the last week. Magnetic resonance imaging scans revealed a large suprasellar cystic lesion with a normal pituitary in the sella turcica. Endocrinological findings showed an extremely high prolactin level of 1250 ng/mL. Because of the sharp decline in vision, the patient underwent total removal of the suprasellar lesion using a transfrontal interhemispheric approach. The tumor pedicle originated in the lower pituitary stalk without any connection to the anterior pituitary gland in the sella turcica, while the diaphragma sellae was incomplete. Clinical and endocrinological cure criteria were fulfilled and postoperative pathology confirmed a prolactin-secreting pituitary adenoma.
CONCLUSION: Ectopic suprasellar pituitary adenomas (ESPAs) are extremely rare intracranial extracerebral tumors. SPAs can be classified into three types according to their origin and their relationship with surrounding tissue. Only type III is theoretically a true ectopic, based on previous reports. Thus, ESPAs are uncommon compared to other EPAs. Our case is the first reported case of a type IIa 'E'SPA and the first description of this subtype classification until now. The pars tuberalis may be different from the pars distalis, and each subtype of adenohypophyseal cells may have different migration characteristics, which leads to different proportions of each hormone-secreting subtype in SPAs and EPAs. Transsphenoidal surgery is minimally invasive, but transcranial surgery may remain a universal option for the treatment of suprasellar lesions.

Abstract
PURPOSE: MCM7 (minichromosome maintenance complex component 7), a DNA replication licensing factor, is a host gene for the oncogenic miR-106b~25 cluster. It has been recently revealed as a relevant prognostic biomarker in a variety of cancers, including pituitary adenomas. The purpose of this study was to assess whether miR-106b~25 and MCM7 levels correlate with tumor invasiveness in a cohort of ACTH-immunopositive adenomas.
METHODS: Tissue samples were obtained intraoperatively from 25 patients with pituitary adenoma. Tumor invasiveness was assessed according to the Knosp grading scale. MCM7, Ki-67 and TP53 levels were assessed by immunohistochemical staining, while the expression of miR-106b-5p, miR-93-5p, miR-93-3p and miR-25-3p were measured using quantitative real-time PCR performed on RNA isolated from FFPE tissues.
RESULTS: We have found a significant increase in MCM7 and Ki-67 labeling indices in invasive ACTHomas. Moreover, MCM7 was ubiquitously overexpressed in Crooke's cell adenomas. The expression of miR-93-5p was significantly elevated in invasive compared to noninvasive tumors. In addition, all four microRNAs from the miR-106b~25 cluster displayed marked upregulation in Crooke's cell adenomas. Remarkably, MCM7 and miR-106b-5p both strongly correlated with Knosp grade. A combination of MCM7 LI and miR-106b~25 cluster expression was able to accurately differentiate invasive from noninvasive tumors and had a significant discriminatory ability to predict postoperative tumor recurrence/progression.
CONCLUSIONS: miR-106b~25 and its host gene MCM7 are potential novel biomarkers for invasive ACTH-immunopositive pituitary adenomas. Additionally, they are both significantly upregulated in rare Crooke's cell adenomas and might therefore contribute to their aggressive phenotype.

Abstract
Background: Myeloid sarcoma (MS) or granulocytic sarcoma is a rare tumor consisting of myeloid blasts with or without maturation occurring at anatomic sites other than the bone marrow. MS can involve any organ system but shows a predilection for skin, bone, and soft tissues of head and neck region. Case report: We report four cases of MS occurring at unusual sites, out of which three were de novo and one was associated with acute myeloid leukemia (AML). Conclusion: Although MS is associated with AML, it can rarely present without any existent hematologic disease. Differential diagnosis of a soft tissue mass should include MS even in the absence of leukemia. Establishment of the correct diagnosis depends on morphologic, histochemical, and immunohistochemical examination.

Abstract
RATIONALE: Ganglioneuroblastoma is usually located in the adrenal gland, retroperitoneal ganglia, or posterior mediastinum, but rarely occurs in the parapharyngeal space.
PATIENT CONCERNS: A 4-year-old girl presented with complaint of progressive inspiratory dyspnea and dysphagia, accompanying left-side Horner's syndrome.
DIAGNOSE: Computed tomography (CT) scan revealed a giant mass with irregular low density in left oropharyngeal and posterior pharyngeal wall. The left carotid artery sheath was pushed to the right. After enhancement, the central part of the mass was strengthened, and the surrounding bones structures appeard normal. Magnetic resonance imaging (MRI) showed a solid mass in the left parapharyngeal space displacing the left carotid sheath posteriorly and laterally. A ganglioneuroblastoma was diagnosed.
INTERVATIONS: The girl was treated by surgery.
OUTCOMES: The postoperative course was uneventful. There was no recurrence was observed during the 1-year follow-up.
LESSONS: The primary cervical ganglioneuroblastoma is rare, we recommended the ganglioneuroblastoma should be considered in the differential diagnosis of a child presenting with a parapharyngeal space mass.

Abstract
A 52-year-old man with intermittent head motor tics, aphasia and right upper extremity weakness had an F-FDG avid left front lobe brain lesion, and a left upper lobe lung lesion on PET/CT. The brain lesion was surgically resected, and was diagnosed pathologically as a histiocytic sarcoma (HS). The lung lesion was also a HS via bronchial biopsy. HS is rare and generally involves nodes as shown on PET/CT in a few case reports. The current case with simultaneous brain and lung HS lesions adds new imaging pattern information of the disease to the literature.

Abstract
Pallister Hall syndrome is autosomal dominant disorder usually diagnosed in infants and children. Current diagnostic criteria include presence of hypothalamic hamartoma, post axial polydactyly and positive family history, but the disease has variable manifestations. Herein we report Pallister Hall syndrome diagnosed in a family where both patients were adults. A 59 year old man developed seizures 4 years prior to our evaluation of him, at which time imaging showed a hypothalamic hamartoma. The seizures were controlled medically. He did well until he had visual changes after a traumatic head injury. Repeat MRI showed slight expansion of the mass with formal visual field testing demonstrating bitemporal hemianopsia. There was no evidence of pituitary dysfunction except for large urine volume. He underwent surgery to debulk the hamartoma and the visual field defects improved. There was no hypopituitarism post-operatively, and the polydyspia resolved. His 29 year old daughter also had seizures and hypothalamic hamartoma. Both patients had had polydactyly with prior surgical correction in childhood. The daughter underwent genetic testing, which revealed a previously undescribed heterozygous single base pair deletion in exon 13 of the GLI3 gene causing a frameshift mutation. Further investigation into family history revealed multiple members in previous generations with polydactyly and/or seizures. Pallister-Hall syndrome is caused by an inherited autosomal dominant or de novo mutation in GLI3 gene. This rare syndrome has not had prevalence defined, however. Generally, diagnoses are made in the pediatric population. Our report adds to the few cases detected in adulthood.

Abstract
BACKGROUND: While major progress has been made in diagnosis and treatment of gliomas based on molecules, molecular features of thalamic glioma have rarely been reported till now.
OBJECTIVE: IDH1 mutation is important for prognosis of gliomas and represents a distinctive category of glioma. We intended to survey specific molecular abnormalities in high-grade thalamic gliomas (WHO III-IV).
METHODS: We collected data of 50 and 93 newly diagnosed high-grade thalamic and superficial glioma patients respectively and conducted a comparative analysis of molecular characteristics between them. We analyzed expressions of molecules as follow: IDH1/2, P53, Ki-67, ATRX, PTEN, MMP9 and MGMT by Immunohistochemistry (IHC). Direct gene sequencing was performed to test the IDH1(R 132H) mutation.
RESULTS: We found a significant difference of IDH1 mutation between those high-grade gliomas, with 92% (46/50) of the thalamic tumors and 71% (66/93) of the superficial gliomas showing IDH1 wild-type (p= 0.004). It also showed that IDH1 mutation in superficial glioblastomas 18.6% (13/70) occurred more than thalamic glioblastomas 2.6% (1/39) (p= 0.017). As to high-grade superficial gliomas, there were 26 patients with IDH1 mutation, which contained 7, 13, and 6 high, moderate and low Ki-67 expression gliomas, respectively. The IDH1 wild-type group (62 patients), was composed of 29, 30, and 3 high, moderate and low Ki-67 expression gliomas, respectively. There was a significant distinction between the IDH1 mutation and Ki-67 expressions (p= 0.024). We also noted that the occurrence of low Ki-67 expressions 23.1% (6/26) in IDH1 mutation group was outnumbered than IDH1 wild-type group 4.8% (3/62) (p= 0.018). In addition, we found PTEN negative correlated with MMP9 negative in thalamic high-grade gliomas, whereas no such difference was found in superficial gliomas (p= 0.016).
CONCLUSION: The rare occurrence of IDH1 mutant high-grade thalamic gliomas strongly suggested that the high-grade thalamic glioma is another distinct tumor entity as compared to the high-grade superficial gliomas.

Abstract
We present a rare case of primary pituitary T cell lymphoma/leukemia (T-LBL) in association with adrenocorticotropic hormone (ACTH) and thyroid stimulating hormone (TSH) expressing pituitary adenoma in a 55-year-old woman highlighting the importance of intra-operative squash smears examination. The patient presented with complaints of headache, diminution of vision and recent onset altered sensorium. MRI revealed a mass lesion in the sellar-suprasellar region with non-visualization of pituitary gland separately, extending to involve adjacent structures diagnosed as invasive pituitary macroadenoma. Intra-operative tissue was sent for squash smear examination. The cytology showed a tumor comprising of sheets of immature lymphoid cells intermixed with clusters of pituitary acinar cells with many mitoses and tingible body macrophages. A diagnosis of presence of immature lymphoid cells within the pituitary was offered and differentials of infiltration by lymphoma cells from systemic disease versus primary central nervous lymphoma-like lymphoma arising in the pituitary adenoma were considered. Later paraffin section examination and immunohistochemistry corroborated with the squash findings and a final diagnosis of primary pituitary T cell lymphoma/leukemia in association with ACTH and TSH expressing pituitary adenoma was made. To date, only six cases of primary pituitary T cell lymphomas, including three T-LBL cases, have been reported. This is the seventh case and first one additionally describing cytohistological correlation and importance of intra-operative cytology.

Abstract
We report here a rare case of symptomatic granular cell tumor (GCT) of the sellar region with a large calcification. A 70-year-old woman presented with a sellar mass, accompanied by bitemporal hemianopia. The patient was diagnosed preoperatively as having a craniopharyngioma or a pituitary adenoma, because of the large calcification. The patient underwent surgical tumor resection via endoscopic trans-sphenoidal surgery and was diagnosed pathologically as having GCT. The patient's postoperative course was uneventful and her visual field disturbance improved soon after the operation. We briefly discuss the pathological discrimination of GCT and other sellar tumors, since there are many unclear points concerning this rare tumor.

Abstract
Gliosarcoma, a variant of isocitrate dehydrogenase-wildtype glioblastoma, is largely a lobar surfacing neoplasm often with dural attachment. In this biphasic neoplasm, the sarcomatous component usually takes the form of fibrosarcoma or malignant fibrous histiocytoma. Heterologous sarcomatous differentiation is a rare phenomenon. Here, we present a case of gliosarcoma with liposarcomatous and myosarcomatous differentiation in a 68-year-old man which was purely intraventricular. This is the first report of such a morphologic pattern in this location. Varied histological components with their immunohistochemical profile are discussed. Of note was the presence of a p53 negative giant cell glioblastoma component, as was the expression in the rest of the tumor.

Abstract
Pediatric high-grade gliomas are rare and occasionally hard to classify. These tumors often feature a well-demarcated histology and are expected to have a better outcome than ordinary diffuse high-grade gliomas in adults. We herein report a case of circumscribed high-grade glioma that showed a distinct molecular profile and followed an excellent course for 26 years. The patient, a 3-year-old boy at onset, presented with a contrast-enhancing mass in the right temporal lobe and underwent resection. Histologically, the tumor mainly consisted of compact bundles of GFAP-positive spindle cells. With its malignant features including brisk mitotic activity and pseudopallisading necrosis, a diagnosis of high-grade astrocytoma was made and adjuvant chemoradiotherapy was administered. After a disease-free period of two decades, the tumor recurred locally. The resected tumor was histologically identical to the primary tumor and additionally contained pleomorphic cells, but lacked eosinophilic granular bodies and reticulin networks. The primary and recurrent tumors both harbored the BRAF V600E mutation, and the recurrent tumor was immunonegative for ATRX. Combined BRAF and ATRX mutations are rare in gliomas, with only a pediatric case of glioblastoma being reported in the literature. However, our case cannot be regarded as glioblastoma because of its well-demarcated histology and excellent course. The distinction of either a diffuse or localized nature in gliomas is important, particularly in children, for predicting prognoses and selecting adjuvant therapies that consequently affect life-long health care. The present case provides novel insights into pediatric high-grade astrocytomas.

Abstract
Pituitary adenoma is one of the most common intracranial neoplasms, usually presenting with endocrinopathies or visual field defects. Granulomatous hypophysitis, one of the subtypes of primary hypophysitis, is a rare neurological entity presenting as a sellar lesion with mass effect symptoms and endocrinological dysfunction. Majority of cases of primary hypophysitis are misdiagnosed as pituitary adenomas preoperatively, and histopathology is required for a definitive diagnosis. Granulomatous hypophysitis can be primary/idiopathic or secondary to a variety of causes such as infection, foreign body reactions, and systemic inflammatory conditions. The presence of tumoral microgranulomas in pituitary adenoma is exceedingly rare, having been documented only twice previously. We present a unique case of recurrent pituitary macroadenoma in a 59-year-old woman with histopathological features of idiopathic intratumoral noncaseating granulomatous inflammation. The presence of a granulomatous response in the absence of a systemic infectious or inflammatory disorder is suggestive of a local tumoral response to an as-yet unidentified antigen.

Abstract
INTRODUCTION: We report the rare finding of recurrent periventricular pseudocysts (PVPC) in consecutive pregnancies in 4 families and their postnatal outcome.
MATERIALS AND METHODS: We reviewed the databases of 3 large ultrasound units searching for the diagnosis of PVPC in 2 pregnancies of the same patient.
RESULTS: The first case of recurrent PVPC was diagnosed in 2011 and since then 3 additional families were diagnosed (8 cases of PVPC all in all). All fetuses underwent fetal MRI that confirmed the presence of frontal or frontocaudal PVPC. Amniocentesis, when performed, demonstrated a normal karyotype. Termination of pregnancy was carried out in 2 pregnancies in 2 of 4 families. The remaining 6 pregnancies ended with a term delivery, and to date all babies are developing normally.
CONCLUSION: The rare finding of recurrent brain PVPC in consecutive pregnancies raises the possibility of a hereditary etiology as opposed to a sporadic event. As in isolated PVPC, frontocaudal 'familial PVPC' appears to carry a favorable prognosis.

Abstract
BACKGROUND: Neoplasms located in Meckel's cave account for 0.2-0.5% of all intracranial tumors. This area is the site of many types of pathological lesions, most often trigeminal nerve schwannomas and meningiomas. Melanin-containing tumors are very rare in this area. These tumor types can be suspected if the MR-characteristics of a tumor has some differences in comparison with other types of central nervous system neoplasms. In fact, differential diagnosis of melanotic tumors is based mainly on the histopathological criteria and immunohistochemical profile. This paper presents a case report of melanotic schwannoma of Meckel's cave and the literature review of the problem.
CASE DESCRIPTION: A 23-year-old man underwent a two-stage surgery due to a dumbbell pigmented mass lesion located in the Meckel's cave. No signs of recurrence were seen on the follow-up MRI 3.5 years after the surgery.
CONCLUSION: Melanin-containing tumor (MCT) can be suspected in presence of radiological characteristics such as a hyperintense MR imaging signal on T1-weighted images and a hypointense signal on T2-weighted images. If a black extracerebral tumor is detected, the main course of surgical treatment is maximal excision despite it may thought to be a malignant melanoma and a temptation to perform partial resection due to unfavorable prognosis. Chemotherapy can be justified in presence of an aggressive melanotic schwannoma.

Abstract
Non-Hodgkin's lymphomas (NHL) with intracranial origin are very rare and constitutes about 1-2% of primary central nervous system lymphomas (PCNSL). Diffuse large B cell lymphoma (DLBCL) is the most common subtype of NHL and mostly seen in immunocompromised patients. Therefore, the occurrence of giant solitary DLBCL in an immunocompetent patient is puzzling. We present a case of 68-year-old man who was admitted at our facility with a history of "hypomnesia of two (2) months" duration. Magnetic resonance imaging (MRI) revealed a space occupying lesion in the bilateral frontal lobe and corpus callosum measuring about 5.4cm * 4.6cm * 3.8cm with mixed signal intensities and vasogenic edema around the mass. Radiological, this mass was mistaken for meningioma until histopathological studies revealed DLBCL. Giant solitary primary intracranial lymphomas are very rare and can be mistake for meningioma even with very experience radiologist or neurosurgeon since the radiological features of PCNSL can be very unspecific. We achieved to total resection because of the giant and solitary nature of our case. The prognosis of PCNSL is general very poor when the patient is immunocompromised. In immunocompetent patients, who are well managed with surgery and chemotherapy, the overall survival and quality of life can very encouraging.

Epidemiology and clinical characteristics of hemangioblastomas in the elderly: An update.

J Clin Neurosci. 2017 Sep;43:264-266

Authors: Yoon JY, Gao A, Das S, Munoz DG

Abstract
Hemangioblastomas are benign tumors, derived from "stromal" cells of unclear origin. While previously thought to be a rare entity in elderly patients, we had previously reported a case series of six elderly patients (aged over 65years at diagnosis) diagnosed with hemangioblastoma in our 12year experience. Here, we report a followup series, describing eight additional cases of hemangioblastomas diagnosed in elderly patients (out of 26 in subjects over age 18). Seven of the eight cases were sporadic, and one case was suspicious for being associated with the von Hippel-Lindau (VHL) syndrome. All eight cases had presented with symptoms related to compression, and surgical resection resulted in good clinical outcomes. Combined with our previous report, 14/103 (13.6%) of the hemangioblastomas diagnosed in a 15-year period in a single hospital were present in elderly patients, suggesting that the prevalence in elderly patients of hemangioblastomas is higher than previously expected.

Abstract
INTRODUCTION: Gliofibromas are rare biphasic tumours with a good prognosis that usually occur in childhood. Rare adult spinal cases have been treated with radiotherapy. This report describes the case of a gliofibroma occurring in a young adult 10 years after treatment for a childhood pilocytic astrocytoma.
CASE: A 14-year-old female underwent complete resection of a right lateral ventricle pilocytic astrocytoma confirmed on postoperative magnetic resonance imaging (MRI). At the age of 17, the tumour recurred, and a second complete resection was performed. Due to the early recurrence, she was placed on long-term MRI surveillance. At the age of 23, an enhancing left midbrain tumour was identified that was suspected to be a recurrent pilocytic astrocytoma. Following surgical resection the histopathology revealed a gliofibroma. Due to the growth of further tumour nodules she was treated with fractionated radiotherapy. There is no disease recurrence after 36 months of follow-up, and the patient remains well.
DISCUSSION: Gliofibromas are tumours which usually occur in childhood; this case report identifies a rare occurrence in an adult. The childhood intraventricular pilocytic astrocytoma was in an anatomically distinct location to the midbrain gliofibroma. Radiotherapy can control these tumours, and follow-up is required to understand the long-term outcome and prognosis.

Abstract
Desmoplastic infantile gliomas are rare, benign tumors of the early infancy period. Two histological subtypes - desmoplastic infantile astrocytoma (DIA) and desmoplastic infantile ganglioglioma - have been described. The characteristic features of DIAs are lobar location, glial histology, and excellent prognosis after complete surgical excision. DIAs usually present as solitary, cortical-surfacing, solid-cystic neoplasms; however, atypical, aggressive, and multifocal variants of DIA have been reported in the literature. These rare DIAs presenting with multiple lesions pose a diagnostic as well as a therapeutic dilemma. We report an unusual case of an 8-month-old female infant diagnosed with multifocal (cranial and spinal) DIA and obstructive hydrocephalus and discuss the radiological and histological features of this rare variant of DIA. The patient underwent ventriculoperitoneal shunt placement to relieve the hydrocephalus, excisional biopsy from a surfacing lesion in the right frontal lobe, and multiple cycles of chemotherapy; however, the lesions continued to progress, and the patient is likely to have an unfavorable outcome.

Patterns of care and treatment outcomes of patients with astroblastoma: a National Cancer Database analysis.

CNS Oncol. 2018 Apr 30;:

Authors: Merfeld EC, Dahiya S, Perkins SM

Abstract
AIM: To evaluate the use of chemotherapy and radiation, and their outcomes for patients with astroblastoma.
PATIENTS & METHODS: This is a retrospective review of patients extracted from the National Cancer Database. We investigated overall survival (OS) using Kaplan-Meier curves. Cox proportional hazards models were used to correlate OS with risk variables and treatments.
RESULTS: OS at 5 years was 79.5%. Patients with high-grade tumors were more likely to receive chemotherapy and radiation. Patients with high-grade astroblastoma who did not receive adjuvant radiation had poor survival.
CONCLUSION: Patients with astroblastoma should be treated with curative intent. Radiation is likely beneficial in high-grade astroblastoma. The exact role of radiation and chemotherapy following surgical resection warrant further investigation.

Abstract
Although neurological features are commonly encountered in Whipple"s disease (WD), presentation with purely neurological patterns is uncommon. Exclusive confinement to the central nervous system (CNS) is extremely rare. In these cases, the development of an isolated cerebral mass is exceptional. In the present paper, the authors describe a case of a 68-year-old man who presented with partial seizures. The neurological examination was normal. The imaging showed a cystic lesion. This tumor-like lesion was removed by performing frontal craniotomy. A histopathological investigation revealed the presence of numerous perivascular foamy histiocytes infiltrating the brain parenchyma. The majority of these histiocytes showed Periodic acid-schiff (PAS)-positive intense staining, which is distinctive feature of cerebral WD. The diagnosis was confirmed by polymerase chain reaction (PCR) analysis of cerebrospinal fluid. There were no gastrointestinal symptoms and no PAS inclusions in intestinal mucosa. The patient received Ceftriaxone intravenously followed by oral trimethoprime-sulfamethoxazole (TMP-SMZ) for 12 months and recovered well. This case illustrates atypical WD, confined exclusively to the central nervous system.

Abstract
Cutaneous angiosarcomas are rare but typically occur in three distinct clinical settings and are most commonly found on the scalp or face of elderly men. Positive prognostic factors include tumor size less than 5 cm, primary tumor location below the head, negative margins after excision, resectability, and younger age. Metastases drastically reduce survival and the most common metastatic site is lung. We present a 43-year-old man who had primary cutaneous angiosarcoma that initially mimicked a furuncle and eventuated in multiple metastases. The metastatic disease included brain involvement, which has rarely been reported, especially in a relatively young person without known predisposing conditions. This unique case also highlights the need for early diagnosis followed by advanced imaging, given the limitations of current therapies and high metastatic potential of angiosarcoma.

A rare case of metastatic extramammary Paget disease of the spine and review of the literature.

J Clin Neurosci. 2017 Nov;45:161-165

Authors: Ahmed AK, Goodwin CR, Abu-Bonsrah N, Nguyen D, Sciubba DM

Abstract
Extramammary Paget disease is an intraepithelial neoplasm affecting cells rich in apocrine glands-often located in the vulvar, scrotal, or perianal region. It typically affects older patients, between the ages of 50 and 80years old, and is most often limited to the epidermis. A 47-year-old Asian male first presented with enlargement of the right inguinal lymph node. A subsequent biopsy revealed extrammamary Paget disease of the scrotum. The patient eventually developed significant worsening back pain with bilateral lower extremity numbness and weakness three months later. Imaging demonstrated a pathologic compression fracture of the L4 vertebral body with metastatic epidural spinal cord compression. The patient underwent surgical decompression of the spine with bilateral L4 laminectomy, resection of epidural tumor, and pedicle screw fixation from L2 to S1. Surgical pathology demonstrated metastatic adenocarcinoma consistent with extramammary Paget disease. Although two other case reports have described spinal metastases from extramammary Paget disease, to the author's knowledge, this represents the first report of surgical decompression and fusion for extramammary Paget disease of the spine.

Abstract
Dermoid cysts can present as a rare, benign, congenital intracranial tumor of neuroectoderm origin trapped during embryogenesis. Past clinical reports have reported lesions in the posterior fossa, at the midline, and in the intradural region all in conjunction with a superficial sinus tract. The authors present a unique patient of a completely intracranial, intradural, dermoid tumor of the midline cerebellum devoid of any evidence of sinus tract. The histological characteristics, radiological features, and management of this unusual patient are described.

Abstract
Myokymia of the tongue is a very rare clinical condition and is much less common than facial or focal myokymia of the limbs. Radiation-induced delayed nerve damage is a well-known cause of myokymia, but other etiologies i.e. tumor recurrence should be considered as a differential diagnosis. We describe a case series of neurophysiologically proven unilateral tongue myokymia, which arose in two patients after radiotherapy of the neck/head and in one patient due to a space occupying meningioma of the cerebrospinal passage affecting the hypoglossal nerve. With this case series and a review of the literature we aim to raise clinical suspicion of tongue myokymia and highlight the clinical and electromyographic impact of myokymia in the diagnosis of malignancies and treatment-associated lesions of the hypoglossal nerve.

Abstract
BACKGROUND: Pineoblastomas are rare, supratentorial, primitive neuroectodermal tumors.
OBJECTIVE: To document outcomes with multimodal therapy and evaluate the impact that the degree of surgical resection has on outcome.
METHODS: A departmental brain tumor database was queried to identify all patients with pathologically proven pineoblastoma who were treated from January 1997 to June 2015 at St. Jude Children's Research Hospital. For each patient, we recorded demographic, pathological, radiological, surgical, and clinical follow-up data. The effect of degree of surgical resection on survival outcomes was analyzed.
RESULTS: Forty-one patients (21 male, 20 female) treated for pineoblastoma were identified. The median age at diagnosis was 5.5 years (range 0.4-28.1) and the median follow-up was 34.5 months. Nineteen patients experienced tumor relapse with a median progression-free survival of 11.3 months, and 18 ultimately succumbed to their disease. Patients who died or experienced treatment failure were younger (median, 2.69 vs 6.5 years, P = .026) and more likely to have metastatic disease at diagnosis (12 [63.2%] vs 5 [22.7%], P = .012). When analyzing only patients 5 years of age or older with focal disease at presentation, those who had a gross total resection or near-total resection-compared with subtotal resection or biopsy-had greater overall survival (75.18 vs 48.57 months), with no patients dying as a result of their cancer.
CONCLUSION: Poor prognostic variables for children with pineoblastoma include young age, metastatic disease at presentation, and tumor relapse. For patients older than 5 years with focal disease, maximal tumor resection should be the goal.